The carrier rate and mutation spectrum of genes associated with hearing loss in South China hearing female population of childbearing age

PubMed Central

2013-01-01

Background Given that hearing loss occurs in 1 to 3 of 1,000 live births and approximately 90 to 95 percent of them are born into hearing families, it is of importance and necessity to get better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the general population. Methods 7,263 unrelated women of childbearing age with normal hearing and without family history of hearing loss were tested with allele-specific PCR-based universal array. Further genetic testing were provided to the spouses of the screened carriers. For those couples at risk, multiple choices were provided, including prenatal diagnosis. Results Among the 7,263 normal hearing participants, 303 subjects carried pathogenic mutations included in the screening chip, which made the carrier rate 4.17%. Of the 303 screened carriers, 282 harbored heterozygous mutated genes associated with autosomal recessive hearing loss, and 95 spouses took further genetic tests. 8 out of the 9 couples harbored deafness-causing mutations in the same gene received prenatal diagnosis. Conclusions Given that nearly 90 to 95 percent of deaf and hard-of-hearing babies are born into hearing families, better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the female population of childbearing age may be of importance in carrier screening and genetic counseling. PMID:23718755

The carrier rate and mutation spectrum of genes associated with hearing loss in South China hearing female population of childbearing age.

PubMed

Yin, Aihua; Liu, Chang; Zhang, Yan; Wu, Jing; Mai, Mingqin; Ding, Hongke; Yang, Jiexia; Zhang, Xiaozhuang

2013-05-29

Given that hearing loss occurs in 1 to 3 of 1,000 live births and approximately 90 to 95 percent of them are born into hearing families, it is of importance and necessity to get better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the general population. 7,263 unrelated women of childbearing age with normal hearing and without family history of hearing loss were tested with allele-specific PCR-based universal array. Further genetic testing were provided to the spouses of the screened carriers. For those couples at risk, multiple choices were provided, including prenatal diagnosis. Among the 7,263 normal hearing participants, 303 subjects carried pathogenic mutations included in the screening chip, which made the carrier rate 4.17%. Of the 303 screened carriers, 282 harbored heterozygous mutated genes associated with autosomal recessive hearing loss, and 95 spouses took further genetic tests. 8 out of the 9 couples harbored deafness-causing mutations in the same gene received prenatal diagnosis. Given that nearly 90 to 95 percent of deaf and hard-of-hearing babies are born into hearing families, better understanding about the carrier rate and mutation spectrum of genes associated with hearing impairment in the female population of childbearing age may be of importance in carrier screening and genetic counseling.

Hearing-loss-associated gene detection in neonatal intensive care unit.

PubMed

Yang, S M; Liu, Ying; Liu, C; Yin, A H; Wu, Y F; Zheng, X E; Yang, H M; Yang, J

2018-02-01

To investigate the frequency and mutation spectrum of hearing loss-associated gene mutation in Neonatal Intensive Care Unit (NICU). Neonates (n=2305) admitted to NICU were enrolled in this study. Nine prominent hearing loss-associated genes, GJB2 (35 del G, 176 del 16,235 del C, 299 del AT), GJB3 (538 C > T), SLC26A4 (IVS7-2A > G, 2168 A > G) and mtDNA 12S rRNA(1555 A > G, 1494 C > T), were detected. There were 73 cases hearing-loss-associated gene mutation among 2305 cases, the mutation frequency was 3.1%, with 40 cases GJB2 (235del C) mutation (54.8%), 6 cases GJB2 (299 del AT) mutation (8.2%), 21 cases SLC26A4 (IVS 7-2 A > G) mutation (28.7%), 4 cases SLC26A4 (2168 A > G) mutation (5.5%), 2 cases of GJB2 (235del C) combined SLC26A4 (IVS 7-2 A > G, 2168 A > G) mutation (2.8%). Among 73 gene mutation cases, preterm neonates presented in 18 cases, accounting for 24.7% (18/73); hyperbilirubinemia in 13 cases, accounting for 17.8% (13/73); Torch Syndrome in 15 cases, with 12 cases CMV, 2 cases rubella, 1 case toxoplasm, respectively, totally accounting for 20.54% (15/73); neonatal pneumonia in 12 cases, accounting for 16.4% (12/73); birth asphyxia in 5 cases, accounting for 6.9% (5/73); sepsis in 5 cases, accounting for 6.9% (5/73); others in 5 cases, accounting for 6.8% (5/73) . The frequency of hearing loss-associated gene mutation was higher in NICU.There were hearing loss-associated gene mutations in the NICU, suggesting this mutation may complicate with perinatal high-risk factors.

No association between apolipoprotein E or N-acetyltransferase 2 gene polymorphisms and age-related hearing loss.

PubMed

Dawes, Piers; Platt, Hazel; Horan, Michael; Ollier, William; Munro, Kevin; Pendleton, Neil; Payton, Antony

2015-01-01

Age-related hearing loss has a genetic component, but there have been limited genetic studies in this field. Both N-acetyltransferase 2 and apolipoprotein E genes have previously been associated. However, these studies have either used small sample sizes, examined a limited number of polymorphisms, or have produced conflicting results. Here we use a haplotype tagging approach to determine association with age-related hearing loss and investigate epistasis between these two genes. Candidate gene association study of a continuous phenotype. We investigated haplotype tagging single nucleotide polymorphisms in the N-acetyltransferase 2 gene and the presence/absence of the apolipoprotein E ε4 allele for association with age-related hearing loss in a cohort of 265 Caucasian elderly volunteers from Greater Manchester, United Kingdom. Hearing phenotypes were generated using principal component analysis of the hearing threshold levels for the better ear (severity, slope, and concavity). Genotype data for the N-acetyltransferase 2 gene was obtained from existing genome-wide association study data from the Illumina 610-Quadv1 chip. Apolipoprotein E genotyping was performed using Sequenom technology. Linear regression analysis was performed using Plink and Stata software. No significant associations (P value, > 0.05) were observed between the N-acetyltransferase 2 or apolipoprotein E gene polymorphisms and any hearing factor. No significant association was observed for epistasis analysis of apolipoprotein E ε4 and the N-acetyltransferase 2 single nucleotide polymorphism rs1799930 (NAT2*6A). We found no evidence to support that either N-acetyltransferase 2 or apolipoprotein E gene polymorphisms are associated with age-related hearing loss in a cohort of 265 elderly volunteers. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

Impact of Gene Patents and Licensing Practices on Access to Genetic Testing for Hearing Loss

PubMed Central

Chandrasekharan, Subhashini; Fiffer, Melissa

2011-01-01

Genetic testing for heritable hearing loss involves a mix of patented and unpatented genes, mutations and testing methods. More than half of all hearing loss is linked to inherited mutations, and five genes are most commonly tested in the United States. There are no patents on three of these genes, but Athena Diagnostics holds exclusive licenses to test for a common mutation in the GJB2 gene associated with about 50% of all cases, as well as mutations in the MTRNR1 gene. This fragmented intellectual property landscape made hearing loss a useful case study for assessing whether patent rights in genetic testing can proliferate or overlap, and whether it is possible to gather the rights necessary to perform testing. Testing for hearing loss is widely available, primarily from academic medical centers. Based on literature reviews and interviews with researchers, research on the genetics of hearing loss has generally not been impeded by patents. There is no consistent evidence of a premium in testing prices attributable to patent status. Athena Diagnostics has, however, used its intellectual property to discourage other providers from offering some tests. There is no definitive answer about the suitability of current patenting and licensing of commonly tested genes because of continuing legal uncertainty about the extent of enforcement of patent rights. Clinicians have also expressed concerns that multiplex tests will be difficult to develop because of overlapping intellectual property and conflict with Athena’s sole provider business model. PMID:20393307

Hearing impairment caused by mutations in two different genes responsible for nonsyndromic and syndromic hearing loss within a single family.

PubMed

Niepokój, Katarzyna; Rygiel, Agnieszka M; Jurczak, Piotr; Kujko, Aleksandra A; Śniegórska, Dominika; Sawicka, Justyna; Grabarczyk, Alicja; Bal, Jerzy; Wertheim-Tysarowska, Katarzyna

2018-02-01

Usher syndrome is rare genetic disorder impairing two human senses, hearing and vision, with the characteristic late onset of vision loss. This syndrome is divided into three types. In all cases, the vision loss is postlingual, while loss of hearing is usually prelingual. The vestibular functions may also be disturbed in Usher type 1 and sometimes in type 3. Vestibular areflexia is helpful in making a proper diagnosis of the syndrome, but, often, the syndrome is misdiagnosed as a nonsyndromic hearing loss. Here, we present a Polish family with hearing loss, which was clinically classified as nonsyndromic. After excluding mutations in the DFNB1 locus, we implemented the next-generation sequencing method and revealed that hearing loss was syndromic and mutations in the USH2A gene indicate Usher syndrome. This research highlights the importance of molecular analysis in establishing a clinical diagnosis of congenital hearing loss.

Parallel evolution of auditory genes for echolocation in bats and toothed whales.

PubMed

Shen, Yong-Yi; Liang, Lu; Li, Gui-Sheng; Murphy, Robert W; Zhang, Ya-Ping

2012-06-01

The ability of bats and toothed whales to echolocate is a remarkable case of convergent evolution. Previous genetic studies have documented parallel evolution of nucleotide sequences in Prestin and KCNQ4, both of which are associated with voltage motility during the cochlear amplification of signals. Echolocation involves complex mechanisms. The most important factors include cochlear amplification, nerve transmission, and signal re-coding. Herein, we screen three genes that play different roles in this auditory system. Cadherin 23 (Cdh23) and its ligand, protocadherin 15 (Pcdh15), are essential for bundling motility in the sensory hair. Otoferlin (Otof) responds to nerve signal transmission in the auditory inner hair cell. Signals of parallel evolution occur in all three genes in the three groups of echolocators--two groups of bats (Yangochiroptera and Rhinolophoidea) plus the dolphin. Significant signals of positive selection also occur in Cdh23 in the Rhinolophoidea and dolphin, and Pcdh15 in Yangochiroptera. In addition, adult echolocating bats have higher levels of Otof expression in the auditory cortex than do their embryos and non-echolocation bats. Cdh23 and Pcdh15 encode the upper and lower parts of tip-links, and both genes show signals of convergent evolution and positive selection in echolocators, implying that they may co-evolve to optimize cochlear amplification. Convergent evolution and expression patterns of Otof suggest the potential role of nerve and brain in echolocation. Our synthesis of gene sequence and gene expression analyses reveals that positive selection, parallel evolution, and perhaps co-evolution and gene expression affect multiple hearing genes that play different roles in audition, including voltage and bundle motility in cochlear amplification, nerve transmission, and brain function.

Prestin-based outer hair cell electromotility in knockin mice does not appear to adjust the operating point of a cilia-based amplifier

PubMed Central

Gao, Jiangang; Wang, Xiang; Wu, Xudong; Aguinaga, Sal; Huynh, Kristin; Jia, Shuping; Matsuda, Keiji; Patel, Manish; Zheng, Jing; Cheatham, MaryAnn; He, David Z.; Dallos, Peter; Zuo, Jian

2007-01-01

The remarkable sensitivity and frequency selectivity of the mammalian cochlea is attributed to a unique amplification process that resides in outer hair cells (OHCs). Although the mammalian-specific somatic motility is considered a substrate of cochlear amplification, it has also been proposed that somatic motility in mammals simply acts as an operating-point adjustment for the ubiquitous stereocilia-based amplifier. To address this issue, we created a mouse model in which a mutation (C1) was introduced into the OHC motor protein prestin, based on previous results in transfected cells. In C1/C1 knockin mice, localization of C1-prestin, as well as the length and number of OHCs, were all normal. In OHCs isolated from C1/C1 mice, nonlinear capacitance and somatic motility were both shifted toward hyperpolarization, so that, compared with WT controls, the amplitude of cycle-by-cycle (alternating, or AC) somatic motility remained the same, but the unidirectional (DC) component reversed polarity near the OHC's presumed in vivo resting membrane potential. No physiological defects in cochlear sensitivity or frequency selectivity were detected in C1/C1 or C1/+ mice. Hence, our results do not support the idea that OHC somatic motility adjusts the operating point of a stereocilia-based amplifier. However, they are consistent with the notion that the AC component of OHC somatic motility plays a dominant role in mammalian cochlear amplification. PMID:17640919

Mutation Analysis of the Common Deafness Genes in Patients with Nonsyndromic Hearing Loss in Linyi by SNPscan Assay.

PubMed

Zhang, Fengguo; Xiao, Yun; Xu, Lei; Zhang, Xue; Zhang, Guodong; Li, Jianfeng; Lv, Huaiqing; Bai, Xiaohui; Wang, Haibo

2016-01-01

Hearing loss is a common sensory disorder, and at least 50% of cases are due to a genetic etiology. Although hundreds of genes have been reported to be associated with nonsyndromic hearing loss, GJB2, SLC26A4, and mtDNA12SrRNA are the major contributors. However, the mutation spectrum of these common deafness genes varies among different ethnic groups. The present work summarized mutations in these three genes and their prevalence in 339 patients with nonsyndromic hearing loss at three different special education schools and one children's hospital in Linyi, China. A new multiplex genetic screening system "SNPscan assay" was employed to detect a total of 115 mutations of the above three genes. Finally, 48.67% of the patients were identified with hereditary hearing loss caused by mutations in GJB2, SLC26A4, and mtDNA12SrRNA. The carrying rate of mutations in the three genes was 37.76%, 19.75%, and 4.72%, respectively. This mutation profile in our study is distinct from other parts of China, with high mutation rate of GJB2 suggesting a unique mutation spectrum in this area.

Hereditary Hearing Loss.

ERIC Educational Resources Information Center

Tran, LenhAnh P.; Grundfast, Kenneth M.

1997-01-01

This article discusses inheritance patterns in hearing loss, epidemiology, clues to genetic causes, locating genes that cause hereditary disorders, genes related to hearing loss disorders in individuals with Usher syndrome, Waardenburg syndrome, Treacher-Collins syndrome, Branchio-oto-renal and Pendred syndromes, and the significance of finding…

Chloride Anions Regulate Kinetics but Not Voltage-Sensor Qmax of the Solute Carrier SLC26a5.

PubMed

Santos-Sacchi, Joseph; Song, Lei

2016-06-07

In general, SLC26 solute carriers serve to transport a variety of anions across biological membranes. However, prestin (SLC26a5) has evolved, now serving as a motor protein in outer hair cells (OHCs) of the mammalian inner ear and is required for cochlear amplification, a mechanical feedback mechanism to boost auditory performance. The mechanical activity of the OHC imparted by prestin is driven by voltage and controlled by anions, chiefly intracellular chloride. Current opinion is that chloride anions control the Boltzmann characteristics of the voltage sensor responsible for prestin activity, including Qmax, the total sensor charge moved within the membrane, and Vh, a measure of prestin's operating voltage range. Here, we show that standard narrow-band, high-frequency admittance measures of nonlinear capacitance (NLC), an alternate representation of the sensor's charge-voltage (Q-V) relationship, is inadequate for assessment of Qmax, an estimate of the sum of unitary charges contributed by all voltage sensors within the membrane. Prestin's slow transition rates and chloride-binding kinetics adversely influence these estimates, contributing to the prevalent concept that intracellular chloride level controls the quantity of sensor charge moved. By monitoring charge movement across frequency, using measures of multifrequency admittance, expanded displacement current integration, and OHC electromotility, we find that chloride influences prestin kinetics, thereby controlling charge magnitude at any particular frequency of interrogation. Importantly, however, this chloride dependence vanishes as frequency decreases, with Qmax asymptoting at a level irrespective of the chloride level. These data indicate that prestin activity is significantly low-pass in the frequency domain, with important implications for cochlear amplification. We also note that the occurrence of voltage-dependent charge movements in other SLC26 family members may be hidden by inadequate

Biophysical Mechanisms Underlying Hearing Loss Associated with a Shortened Tectorial Membrane

NASA Astrophysics Data System (ADS)

Oghalai, John S.; Xia, Anping; Liu, Christopher C.; Gao, Simon S.; Applegate, Brian E.; Puria, Sunil; Rousso, Itay; Steele, Charles

2011-11-01

The tectorial membrane (TM) connects to the stereociliary bundles of outer hair cells (OHCs). Herein, we summarize key experimental data and modeling analyses that describe how biophysical alterations to these connections underlie hearing loss. The heterozygous C1509G mutation in alpha tectorin produces partial congenital hearing loss that progresses in humans. We engineered this mutation in mice, and histology revealed that the TM was shortened. DIC imaging of freshly-dissected cochlea as well as imaging with optical coherence tomography indicated that the TM is malformed and only stimulates the first row of OHCs. Noise exposure produced acute threshold shifts that fully recovered in Tecta+/+ mice although there was some OHC loss within all three rows at the cochlear base. In contrast, threshold shifts only partially recovered in TectaC1509G/+ mice. This was associated with OHC loss more apically and nearly entirely within the first row. Young's modulus of the TM, measured using atomic force microscopy, was substantially reduced at the middle and basal regions. Both the wild-type and heterozygous conditions were simulated in a computational model. This demonstrated that the normalized stress distribution levels between the TM and the tall cilia were significantly elevated in the middle region of the heterozygous cochlea. Another feature of the TectaC1509G/+ mutation is higher prestin expression within all three rows of OHCs. This increased electricallyevoked movements of the reticular lamina and otoacoustic emissions. Furthermore, electrical stimulation was associated with an increased risk of OHC death as measured by vital dye staining. Together, these findings indicate that uncoupling of the TM from some OHCs not only leads to partial hearing loss, but also puts the OHCs that remain coupled at higher risk. Both the mechanics of the malformed TM and increased electromotility contribute to this higher risk profile.

[Application of MALDI-TOF-MS in gene testing for non-syndromic hearing loss].

PubMed

Zeng, Yun; Jiang, Dan; Feng, Da-fei; Jin, Dong-dong; Wu, Xiao-hui; Ding, Yan-li; Zou, Jing

2013-12-01

To investigate the feasibility of Matrix-Assisted Laser Desorption-Ionization Time of Flight Mass Spectrometry (MALDI-TOF-MS) , according to the genetic test of non-syndromic hearing loss (NSHL), and check using the direct sequencing. Peripheral blood was collected from 454 NSHL patients. DNA samples were extracted and 20 loci of the four common disease-causing genes were analysed by MALDI-TOF-MS, including GJB2 (35delG, 167delT, 176_191del16, 235delC, 299_300delAT ), GJB3 (538C→T, 547G→A), SLC26A4 (281C→T, 589G→A, IVS7-2A→G, 1174A→T, 1226G→A, 1229C→T, IVS15+5G→A, 1975G→C, 2027T→A, 2162C→T, 2168A→G), and mitochondrial 12S rRNA (1494C→T, 1555A→G). Direct sequencing was also used to analyse the aforementioned 20 loci in order to validate the accuracy of MALDI-TOF-MS. Among the 454 patients, 166 cases (36.56%) of disease-causing mutations were detected, which included 69 cases (21.15%) of GJB2 gene mutation, four cases (0.88%) of GJB3 gene mutation, 64 cases (14.10%) of SLC26A4 gene mutation, and three cases (0.66%) of mitochondrial 12S rRNA gene mutation. Moreover, the results obtained from direct sequencing and MALDI-TOF-MS were consistent, and the results showed that the two methods were consistent. The MALDI-TOF-MS detection method was designed based on the hearing loss-related mutation hotspots seen in the Chinese population, and it has a high detection rate for NSHL related mutations. In comparison to the conventional detection methods, MALDI-TOF-MS has the following advantages: more detection sites, greater coverage, accurate, high throughput and low cost. Therefore, this method is capable of satisfying the needs of clinical detection for hearing impairment and it is suitable for large-scale implementation.

Missense mutation in the USH2A gene: association with recessive retinitis pigmentosa without hearing loss.

PubMed

Rivolta, C; Sweklo, E A; Berson, E L; Dryja, T P

2000-06-01

Microdeletions Glu767(1-bp del), Thr967(1-bp del), and Leu1446(2-bp del) in the human USH2A gene have been reported to cause Usher syndrome type II, a disorder characterized by retinitis pigmentosa (RP) and mild-to-severe hearing loss. Each of these three frameshift mutations is predicted to lead to an unstable mRNA transcript that, if translated, would result in a truncated protein lacking the carboxy terminus. Here, we report Cys759Phe, a novel missense mutation in this gene that changes an amino-acid residue within the fifth laminin-epidermal growth factor-like domain of the USH2A gene and that is associated with recessive RP without hearing loss. This single mutation was found in 4.5% of 224 patients with recessive RP, suggesting that USH2A could cause more cases of nonsyndromic recessive RP than does any other gene identified to date.

Sex-specific predictors of hearing-aid use in older persons: The age, gene/environment susceptibility - Reykjavik study

PubMed Central

Fisher, Diana E.; Li, Chuan-Ming; Hoffman, Howard J.; Chiu, May S.; Themann, Christa L.; Petersen, Hannes; Jonsson, Palmi V.; Jonsson, Helgi; Jonasson, Fridbert; Sverrisdottir, Johanna Eyrun; Launer, Lenore J.; Eiriksdottir, Gudny; Gudnason, Vilmundur; Cotch, Mary Frances

2015-01-01

Objective We estimate the prevalence of hearing-aid use in Iceland and identify sex-specific factors associated with use. Design Population-based cohort study. Study sample A total of 5172 age, gene/environment susceptibility - Reykjavik study (AGES-RS) participants, aged 67 to 96 years (mean age 76.5 years), who completed air-conduction and pure-tone audiometry. Results Hearing-aid use was reported by 23.0% of men and 15.9% of women in the cohort, although among participants with at least moderate hearing loss in the better ear (pure-tone average [PTA] of thresholds at 0.5, 1, 2, and 4 kHz ≥ 35 dB hearing level [HL]) it was 49.9% and did not differ by sex. Self-reported hearing loss was the strongest predictor of hearing-aid use in men [OR: 2.68 (95% CI: 1.77, 4.08)] and women [OR: 3.07 (95% CI: 1.94, 4.86)], followed by hearing loss severity based on audiometry. Having diabetes or osteoarthritis were significant positive predictors of use in men, whereas greater physical activity and unimpaired cognitive status were important in women. Conclusions Hearing-aid use was comparable in Icelandic men and women with moderate or greater hearing loss. Self-recognition of hearing loss was the factor most predictive of hearing-aid use; other influential factors differed for men and women. PMID:25816699

Novel compound heterozygous mutations in MYO7A gene associated with autosomal recessive sensorineural hearing loss in a Chinese family.

PubMed

Ma, Yalin; Xiao, Yun; Zhang, Fengguo; Han, Yuechen; Li, Jianfeng; Xu, Lei; Bai, Xiaohui; Wang, Haibo

2016-04-01

Mutations in MYO7A gene have been reported to be associated with Usher Syndrome type 1B (USH1B) and nonsyndromic hearing loss (DFNB2, DFNA11). Most mutations in MYO7A gene caused USH1B, whereas only a few reported mutations led to DFNB2 and DFNA11. The current study was designed to investigate the mutations among a Chinese family with autosomal recessive hearing loss. In this study, we present the clinical, genetic and molecular characteristics of a Chinese family. Targeted capture of 127 known deafness genes and next-generation sequencing were employed to study the genetic causes of two siblings in the Chinese family. Sanger sequencing was employed to examine those variant mutations in the members of this family and other ethnicity-matched controls. We identified the novel compound heterozygous mutant alleles of MYO7A gene: a novel missense mutation c.3671C>A (p.A1224D) and a reported insert mutation c.390_391insC (p.P131PfsX9). Variants were further confirmed by Sanger sequencing. These two compound heterozygous variants were co-segregated with autosomal recessive hearing loss phenotype. The gene mutation analysis and protein sequence alignment further supported that the novel compound heterozygous mutations were pathogenic. The novel compound heterozygous mutations (c.3671C>A and c.390_391insC) in MYO7A gene identified in this study were responsible for the autosomal recessive sensorineural hearing loss of this Chinese family. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

The Bjornstad syndrome (sensorineural hearing loss and pili torti) disease gene maps to chromosome 2q34-36.

PubMed Central

Lubianca Neto, J F; Lu, L; Eavey, R D; Flores, M A; Caldera, R M; Sangwatanaroj, S; Schott, J J; McDonough, B; Santos, J I; Seidman, C E; Seidman, J G

1998-01-01

We report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at theta = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome. PMID:9545407

Potential treatments for genetic hearing loss in humans: current conundrums.

PubMed

Minoda, R; Miwa, T; Ise, M; Takeda, H

2015-08-01

Genetic defects are a major cause of hearing loss in newborns. Consequently, hearing loss has a profound negative impact on human daily living. Numerous causative genes for genetic hearing loss have been identified. However, presently, there are no truly curative treatments for this condition. There have been several recent reports on successful treatments in mice using embryonic gene therapy, neonatal gene therapy and neonatal antisense oligonucleotide therapy. Herein, we describe state-of-the-art research on genetic hearing loss treatment through gene therapy and discuss the obstacles to overcome in curative treatments of genetic hearing loss in humans.

An overview of hereditary hearing loss.

PubMed

Bayazit, Yildirim A; Yilmaz, Metin

2006-01-01

Understanding the genetic basis of hearing loss is important because almost 50% of profound hearing loss are caused by genetic factors and more than 120 independent genes have been identified. In this review, after a brief explanation of some genetic terms (allele, heterozygosis, homozygosis, polymorphism, genotype and phenotype), classification of genetic hearing loss (syndromic versus nonsyndromic, and recessive dominant, X-linked and mitochondrial) was performed. Some of the most common syndromes (Usher, Pendred, Jervell and Lange-Nielsen, Waardenburg, branchio-oto-renal, Stickler, Treacher Collins and Alport syndromes, biotinidase deficiency and Norrie disease) causing genetic hearing loss were also explained briefly. The genes involved in hearing loss and genetic heterogeneity were presented. Copyright 2006 S. Karger AG, Basel.

Molecular biology of hearing

PubMed Central

Stöver, Timo; Diensthuber, Marc

2012-01-01

The inner ear is our most sensitive sensory organ and can be subdivided into three functional units: organ of Corti, stria vascularis and spiral ganglion. The appropriate stimulus for the organ of hearing is sound, which travels through the external auditory canal to the middle ear where it is transmitted to the inner ear. The inner ear houses the hair cells, the sensory cells of hearing. The inner hair cells are capable of mechanotransduction, the transformation of mechanical force into an electrical signal, which is the basic principle of hearing. The stria vascularis generates the endocochlear potential and maintains the ionic homeostasis of the endolymph. The dendrites of the spiral ganglion form synaptic contacts with the hair cells. The spiral ganglion is composed of neurons that transmit the electrical signals from the cochlea to the central nervous system. In recent years there has been significant progress in research on the molecular basis of hearing. An increasing number of genes and proteins related to hearing are being identified and characterized. The growing knowledge of these genes contributes not only to greater appreciation of the mechanism of hearing but also to a deeper understanding of the molecular basis of hereditary hearing loss. This basic research is a prerequisite for the development of molecular diagnostics and novel therapies for hearing loss. PMID:22558056

Novel sequence variants in the TMIE gene in families with autosomal recessive nonsyndromic hearing impairment

PubMed Central

Santos, Regie Lyn P.; El-Shanti, Hatem; Sikandar, Shaheen; Lee, Kwanghyuk; Bhatti, Attya; Yan, Kai; Chahrour, Maria H.; McArthur, Nathan; Pham, Thanh L.; Mahasneh, Amjad Abdullah; Ahmad, Wasim

2010-01-01

To date, 37 genes have been identified for nonsyndromic hearing impairment (NSHI). Identifying the functional sequence variants within these genes and knowing their population-specific frequencies is of public health value, in particular for genetic screening for NSHI. To determine putatively functional sequence variants in the transmembrane inner ear (TMIE) gene in Pakistani and Jordanian families with autosomal recessive (AR) NSHI, four Jordanian and 168 Pakistani families with ARNSHI that is not due to GJB2 (CX26) were submitted to a genome scan. Two-point and multipoint parametric linkage analyses were performed, and families with logarithmic odds (LOD) scores of 1.0 or greater within the TMIE region underwent further DNA sequencing. The evolutionary conservation and location in predicted protein domains of amino acid residues where sequence variants occurred were studied to elucidate the possible effects of these sequence variants on function. Of seven families that were screened for TMIE, putatively functional sequence variants were found to segregate with hearing impairment in four families but were not seen in not less than 110 ethnically matched control chromosomes. The previously reported c.241C>T (p.R81C) variant was observed in two Pakistani families. Two novel variants, c.92A>G (p.E31G) and the splice site mutation c.212–2A>C, were identified in one Pakistani and one Jordanian family, respectively. The c.92A>G (p.E31G) variant occurred at a residue that is conserved in the mouse and is predicted to be extracellular. Conservation and potential functionality of previously published mutations were also examined. The prevalence of functional TMIE variants in Pakistani families is 1.7% [95% confidence interval (CI) 0.3–4.8]. Further studies on the spectrum, prevalence rates, and functional effect of sequence variants in the TMIE gene in other populations should demonstrate the true importance of this gene as a cause of hearing impairment. PMID:16389551

A Point Mutation in the Gene for Asparagine-Linked Glycosylation 10B (Alg10b) Causes Nonsyndromic Hearing Impairment in Mice (Mus musculus)

PubMed Central

Probst, Frank J.; Corrigan, Rebecca R.; del Gaudio, Daniela; Salinger, Andrew P.; Lorenzo, Isabel; Gao, Simon S.; Chiu, Ilene; Xia, Anping

2013-01-01

The study of mouse hearing impairment mutants has led to the identification of a number of human hearing impairment genes and has greatly furthered our understanding of the physiology of hearing. The novel mouse mutant neurological/sensory 5 (nse5) demonstrates a significantly reduced or absent startle response to sound and is therefore a potential murine model of human hearing impairment. Genetic analysis of 500 intercross progeny localized the mutant locus to a 524 kilobase (kb) interval on mouse chromosome 15. A missense mutation in a highly-conserved amino acid was found in the asparagine-linked glycosylation 10B gene (Alg10b), which is within the critical interval for the nse5 mutation. A 20.4 kb transgene containing a wildtype copy of the Alg10b gene rescued the mutant phenotype in nse5/nse5 homozygous animals, confirming that the mutation in Alg10b is responsible for the nse5/nse5 mutant phenotype. Homozygous nse5/nse5 mutants had abnormal auditory brainstem responses (ABRs), distortion product otoacoustic emissions (DPOAEs), and cochlear microphonics (CMs). Endocochlear potentials (EPs), on the other hand, were normal. ABRs and DPOAEs also confirmed the rescue of the mutant nse5/nse5 phenotype by the wildtype Alg10b transgene. These results suggested a defect in the outer hair cells of mutant animals, which was confirmed by histologic analysis. This is the first report of mutation in a gene involved in the asparagine (N)-linked glycosylation pathway causing nonsyndromic hearing impairment, and it suggests that the hearing apparatus, and the outer hair cells in particular, are exquisitely sensitive to perturbations of the N-linked glycosylation pathway. PMID:24303013

A dominant variant in the PDE1C gene is associated with nonsyndromic hearing loss.

PubMed

Wang, Li; Feng, Yong; Yan, Denise; Qin, Litao; Grati, M'hamed; Mittal, Rahul; Li, Tao; Sundhari, Abhiraami Kannan; Liu, Yalan; Chapagain, Prem; Blanton, Susan H; Liao, Shixiu; Liu, Xuezhong

2018-06-02

Identification of genes with variants causing non-syndromic hearing loss (NSHL) is challenging due to genetic heterogeneity. The difficulty is compounded by technical limitations that in the past prevented comprehensive gene identification. Recent advances in technology, using targeted capture and next-generation sequencing (NGS), is changing the face of gene identification and making it possible to rapidly and cost-effectively sequence the whole human exome. Here, we characterize a five-generation Chinese family with progressive, postlingual autosomal dominant nonsyndromic hearing loss (ADNSHL). By combining population-specific mutation arrays, targeted deafness genes panel, whole exome sequencing (WES), we identified PDE1C (Phosphodiesterase 1C) c.958G>T (p.A320S) as the disease-associated variant. Structural modeling insights into p.A320S strongly suggest that the sequence alteration will likely affect the substrate-binding pocket of PDE1C. By whole-mount immunofluorescence on postnatal day 3 mouse cochlea, we show its expression in outer (OHC) and inner (IHC) hair cells cytosol co-localizing with Lamp-1 in lysosomes. Furthermore, we provide evidence that the variant alters the PDE1C hydrolytic activity for both cyclic adenosine monophosphate (cAMP) and cyclic guanosine monophosphate (cGMP). Collectively, our findings indicate that the c.958G>T variant in PDE1C may disrupt the cross talk between cGMP-signaling and cAMP pathways in Ca 2+ homeostasis.

Role of DFNB1 mutations in hereditary hearing loss among assortative mating hearing impaired families from South India.

PubMed

Amritkumar, Pavithra; Jeffrey, Justin Margret; Chandru, Jayasankaran; Vanniya S, Paridhy; Kalaimathi, M; Ramakrishnan, Rajagopalan; Karthikeyen, N P; Srikumari Srisailapathy, C R

2018-06-19

DFNB1, the first locus to have been associated with deafness, has two major genes GJB2 & GJB6, whose mutations have played vital role in hearing impairment across many ethnicities in the world. In our present study we have focused on the role of these mutations in assortative mating hearing impaired families from south India. One hundred and six assortatively mating hearing impaired (HI) families of south Indian origin comprising of two subsets: 60 deaf marrying deaf (DXD) families and 46 deaf marrying normal hearing (DXN) families were recruited for this study. In the 60 DXD families, 335 members comprising of 118 HI mates, 63 other HI members and 154 normal hearing members and in the 46 DXN families, 281 members comprising of 46 HI and their 43 normal hearing partners, 50 other HI members and 142 normal hearing family members, participated in the molecular study. One hundred and sixty five (165) healthy normal hearing volunteers were recruited as controls for this study. All the participating members were screened for variants in GJB2 and GJB6 genes and the outcome of gene mutations were compared in the subsequent generation in begetting deaf offspring. The DFNB1 allele frequencies for DXD mates and their offspring were 36.98 and 38.67%, respectively and for the DXN mates and their offspring were 22.84 and 24.38%, respectively. There was a 4.6% increase in the subsequent generation in the DXD families, while a 6.75% increase in the DXN families, which demonstrates the role of assortative mating along with consanguinity in the increase of DFNB1 mutations in consecutive generations. Four novel variants, p.E42D (in GJB2 gene), p.Q57R, p.E101Q, p.R104H (in GJB6 gene) were also identified in this study. This is the first study from an Indian subcontinent reporting novel variants in the coding region of GJB6 gene. This is perhaps the first study in the world to test real-time, the hypothesis proposed by Nance et al. in 2000 (intense phenotypic assortative mating

A genetically-encoded YFP sensor with enhanced chloride sensitivity, photostability and reduced ph interference demonstrates augmented transmembrane chloride movement by gerbil prestin (SLC26a5).

PubMed

Zhong, Sheng; Navaratnam, Dhasakumar; Santos-Sacchi, Joseph

2014-01-01

Chloride is the major anion in cells, with many diseases arising from disordered Cl- regulation. For the non-invasive investigation of Cl- flux, YFP-H148Q and its derivatives chameleon and Cl-Sensor previously were introduced as genetically encoded chloride indicators. Neither the Cl- sensitivity nor the pH-susceptibility of these modifications to YFP is optimal for precise measurements of Cl- under physiological conditions. Furthermore, the relatively poor photostability of YFP derivatives hinders their application for dynamic and quantitative Cl- measurements. Dynamic and accurate measurement of physiological concentrations of chloride would significantly affect our ability to study effects of chloride on cellular events. In this study, we developed a series of YFP derivatives to remove pH interference, increase photostability and enhance chloride sensitivity. The final product, EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP), has a chloride Kd of 14 mM and pKa of 5.9. The bleach time constant of 175 seconds is over 15-fold greater than wild-type EYFP. We have used the sensor fused to the transmembrane protein prestin (gerbil prestin, SLC26a5), and shown for the first time physiological (mM) chloride flux in HEK cells expressing this protein. This modified fluorescent protein will facilitate investigations of dynamics of chloride ions and their mediation of cell function. Modifications to YFP (EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP) results in a photostable fluorescent protein that allows measurement of physiological changes in chloride concentration while remaining minimally affected by changes in pH.

Genetic testing for hearing impairment.

PubMed

Topsakal, V; Van Camp, G; Van de Heyning, P

2005-01-01

For some patients, genetic testing can reveal the etiology of their hearing impairment, and can provide evidence for a medical diagnosis. However, a gap between fundamental genetic research on hereditary deafness and clinical otology emerges because of the steadily increasing number of discovered genes for hereditary hearing impairment (HHI) and the comparably low clinical differentiation of the HHIs. In an attempt to keep up with the scientific progress, this article enumerates the indications of genetic testing for HHI from a clinical point of view and describes the most frequently encountered HHIs in Belgium. Domains of recent scientific interest, molecular biological aspects, and some pitfalls with HHIs are highlighted. The overview comprises bilateral congenital hearing loss, late-onset progressive high frequency hearing loss, progressive bilateral cochleo-vestibular deficit, and progressive low frequency hearing loss. Also, several syndromal forms of HHI are summarized, and the availability of genetic tests mentioned. Finally, the requirements for successful linkage analysis, an important genetic research tool for localizing the potential genes of a trait on a chromosome, are briefly described.

AUDIOME: a tiered exome sequencing-based comprehensive gene panel for the diagnosis of heterogeneous nonsyndromic sensorineural hearing loss.

PubMed

Guan, Qiaoning; Balciuniene, Jorune; Cao, Kajia; Fan, Zhiqian; Biswas, Sawona; Wilkens, Alisha; Gallo, Daniel J; Bedoukian, Emma; Tarpinian, Jennifer; Jayaraman, Pushkala; Sarmady, Mahdi; Dulik, Matthew; Santani, Avni; Spinner, Nancy; Abou Tayoun, Ahmad N; Krantz, Ian D; Conlin, Laura K; Luo, Minjie

2018-03-29

PurposeHereditary hearing loss is highly heterogeneous. To keep up with rapidly emerging disease-causing genes, we developed the AUDIOME test for nonsyndromic hearing loss (NSHL) using an exome sequencing (ES) platform and targeted analysis for the curated genes.MethodsA tiered strategy was implemented for this test. Tier 1 includes combined Sanger and targeted deletion analyses of the two most common NSHL genes and two mitochondrial genes. Nondiagnostic tier 1 cases are subjected to ES and array followed by targeted analysis of the remaining AUDIOME genes.ResultsES resulted in good coverage of the selected genes with 98.24% of targeted bases at >15 ×. A fill-in strategy was developed for the poorly covered regions, which generally fell within GC-rich or highly homologous regions. Prospective testing of 33 patients with NSHL revealed a diagnosis in 11 (33%) and a possible diagnosis in 8 cases (24.2%). Among those, 10 individuals had variants in tier 1 genes. The ES data in the remaining nondiagnostic cases are readily available for further analysis.ConclusionThe tiered and ES-based test provides an efficient and cost-effective diagnostic strategy for NSHL, with the potential to reflex to full exome to identify causal changes outside of the AUDIOME test.Genetics in Medicine advance online publication, 29 March 2018; doi:10.1038/gim.2018.48.

Genetic Effects on Sensorineural Hearing Loss and Evidence-based Treatment for Sensorineural Hearing Loss.

PubMed

Yu, Yong-qiang; Yang, Huai-an; Xiao, Ming; Wang, Jing-wei; Huang, Dong-yan; Bhambhani, Yagesh; Sonnenberg, Lyn; Clark, Brenda; Jin, Yuan-zhe; Fu, Wei-neng; Zhang, Jie; Yu, Qian; Liang, Xue-ting; Zhang, Ming

2015-09-01

In this article, the mechanism of inheritance behind inherited hearing loss and genetic susceptibility in noise-induced hearing loss are reviewed. Conventional treatments for sensorineural hearing loss (SNHL), i.e. hearing aid and cochlear implant, are effective for some cases, but not without limitations. For example, they provide little benefit for patients of profound SNHL or neural hearing loss, especially when the hearing loss is in poor dynamic range and with low frequency resolution. We emphasize the most recent evidence-based treatment in this field, which includes gene therapy and allotransplantation of stem cells. Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss. Although some treatments are still at the experimental stage, it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.

A Genetically-Encoded YFP Sensor with Enhanced Chloride Sensitivity, Photostability and Reduced pH Interference Demonstrates Augmented Transmembrane Chloride Movement by Gerbil Prestin (SLC26a5)

PubMed Central

Zhong, Sheng; Navaratnam, Dhasakumar; Santos-Sacchi, Joseph

2014-01-01

Background Chloride is the major anion in cells, with many diseases arising from disordered Cl− regulation. For the non-invasive investigation of Cl− flux, YFP-H148Q and its derivatives chameleon and Cl-Sensor previously were introduced as genetically encoded chloride indicators. Neither the Cl− sensitivity nor the pH-susceptibility of these modifications to YFP is optimal for precise measurements of Cl− under physiological conditions. Furthermore, the relatively poor photostability of YFP derivatives hinders their application for dynamic and quantitative Cl− measurements. Dynamic and accurate measurement of physiological concentrations of chloride would significantly affect our ability to study effects of chloride on cellular events. Methodology/Principal Findings In this study, we developed a series of YFP derivatives to remove pH interference, increase photostability and enhance chloride sensitivity. The final product, EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP), has a chloride Kd of 14 mM and pKa of 5.9. The bleach time constant of 175 seconds is over 15-fold greater than wild-type EYFP. We have used the sensor fused to the transmembrane protein prestin (gerbil prestin, SLC26a5), and shown for the first time physiological (mM) chloride flux in HEK cells expressing this protein. This modified fluorescent protein will facilitate investigations of dynamics of chloride ions and their mediation of cell function. Conclusions Modifications to YFP (EYFP-F46L/Q69K/H148Q/I152L/V163S/S175G/S205V/A206K (monomeric Cl-YFP) results in a photostable fluorescent protein that allows measurement of physiological changes in chloride concentration while remaining minimally affected by changes in pH. PMID:24901231

Cytokine gene polymorphism associations with congenital cytomegalovirus infection and sensorineural hearing loss.

PubMed

Kasztelewicz, B; Czech-Kowalska, J; Lipka, B; Milewska-Bobula, B; Borszewska-Kornacka, M K; Romańska, J; Dzierżanowska-Fangrat, K

2017-10-01

Cytomegalovirus (CMV) is the most common viral agent of congenital infections and a leading nongenetic cause of sensorineural hearing loss (SNHL). The host immunologic factors that render a developing foetus prone to intrauterine CMV infection and development of hearing loss are unknown. The aim of this study was to assess the potential associations between the polymorphisms within cytokine and cytokine receptors genes, and the risk of congenital CMV infection, and the hearing outcome. A panel of 11 candidate single nucleotide polymorphisms (SNPs): TNF rs1799964, TNF rs1800629, TNFRSF1A rs4149570, IL1B rs16944, IL1B rs1143634, IL10 rs1800896, IL10RA rs4252279, IL12B rs3212227, CCL2 rs1024611, CCL2 rs13900, CCR5 rs333 was genotyped in 470 infants (72 with confirmed intrauterine CMV infection and 398 uninfected controls), and related to congenital CMV infection, and the outcome. In multivariate analysis, the IL1B rs16944 TT and TNF rs1799964 TC genotypes were significantly associated with intrauterine CMV infection (aOR = 2.32; 95% CI, 1.11-4.89; p = 0.032, and aOR = 2.17, 95% CI, 1.25-3.77; p = 0.007, respectively). Twenty-two out of 72 congenitally infected newborns had confirmed SNHL. Carriers of CT or TT genotype of CCL2 rs13900 had increased risk of hearing loss at birth and at 6 months of age (aOR = 3.59; p = 0.028 and aOR = 4.10; p = 0.039, respectively). This is the first study to report an association between SNPs in IL1B, TNF, and CCL2, and susceptibility to congenital CMV infection (IL1B and TNF) and SNHL (CCL2).

PAX3 gene deletion detected by microarray analysis in a girl with hearing loss.

PubMed

Drozniewska, Malgorzata; Haus, Olga

2014-01-01

Deletions of the PAX3 gene have been rarely reported in the literature. Mutations of this gene are a common cause of Waardenburg syndrome type 1 and 3. We report a 16 year old female presenting hearing loss and normal intellectual development, without major features of Waardenburg syndrome type 1, and without family history of the syndrome. Her phenotype, however, overlaps with features of craniofacial-deafness-hand syndrome. Microarray analysis showed ~862 kb de novo deletion at 2q36.1 including PAX3. The above findings suggest that the rearrangement found in our patient appeared de novo and with high probability is a cause of her phenotype.

Hearing regulates Drosophila aggression.

PubMed

Versteven, Marijke; Vanden Broeck, Lies; Geurten, Bart; Zwarts, Liesbeth; Decraecker, Lisse; Beelen, Melissa; Göpfert, Martin C; Heinrich, Ralf; Callaerts, Patrick

2017-02-21

Aggression is a universal social behavior important for the acquisition of food, mates, territory, and social status. Aggression in Drosophila is context-dependent and can thus be expected to involve inputs from multiple sensory modalities. Here, we use mechanical disruption and genetic approaches in Drosophila melanogaster to identify hearing as an important sensory modality in the context of intermale aggressive behavior. We demonstrate that neuronal silencing and targeted knockdown of hearing genes in the fly's auditory organ elicit abnormal aggression. Further, we show that exposure to courtship or aggression song has opposite effects on aggression. Our data define the importance of hearing in the control of Drosophila intermale aggression and open perspectives to decipher how hearing and other sensory modalities are integrated at the neural circuit level.

Genetics of non syndromic hearing loss.

PubMed

Venkatesh, M D; Moorchung, Nikhil; Puri, Bipin

2015-10-01

Non Syndromic Hearing Loss is an important cause for hearing loss. One in 1000 newborns have some hearing impairment. Over 400 genetic syndromes have been described. Non Syndromic Hearing Loss (NSHL) can be inherited in an Autosomal Dominant, Autosomal Recessive or a Sex Linked fashion. There are several reasons why genetic testing should be done in cases of NSHL, the main reasons being for genetic screening and for planning treatment. This review describes the genes involved in NSHL and the genetic mechanisms involved in the pathogenesis of the disease.

Progressive hearing loss and degeneration of hair cell stereocilia in taperin gene knockout mice

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chen, Mo; Wang, Qin; Zhu, Gang-Hua

The TPRN gene encodes taperin, which is prominently present at the taper region of hair cell stereocilia. Mutations in TPRN have been reported to cause autosomal recessive nonsyndromic deafness 79(DFNB 79). To investigate the role of taperin in pathogenesis of hearing loss, we generated TPRN knockout mice using TALEN technique. Sanger sequencing confirmed an 11 bp deletion at nucleotide 177–187 in exon 1 of TPRN, which results in a truncated form of taperin protein. Heterozygous TPRN{sup +/−} mice showed apparently normal auditory phenotypes to their wide-type (WT) littermates. Homozygous TPRN{sup −/−} mice exhibited progressive sensorineural hearing loss as reflected bymore » auditory brainstem response to both click and tone burst stimuli at postnatal days 15 (P15), 30 (P30), and 60 (P60). Alex Fluor-594 phalloidin labeling showed no obvious difference in hair cell numbers in the cochlea between TPRN{sup −/−} mice and WT mice under light microscope. However, scanning electronic microscopy revealed progressive degeneration of inner hair cell stereocilia, from apparently normal at postnatal days 3 (P3) to scattered absence at P15 and further to substantial loss at P30. The outer hair cell stereocilia also showed progressive degeneration, though much less severe, Collectively, we conclude that taperin plays an important role in maintenance of hair cell stereocilia. Establishment of TPRN knockout mice enables further investigation into the function of this gene. - Highlights: • TPRN{sup −/−} mice were generated using TALEN technique. • TPRN{sup −/−} mice presented progressive hearing loss. • WT and TPRN{sup −/−} mice showed no difference in hair cell numbers. • TPRN{sup −/−} mice showed progressive degeneration of hair cell stereocilia.« less

The Genetic Architecture of Noise-Induced Hearing Loss: Evidence for a Gene-by-Environment Interaction.

PubMed

Lavinsky, Joel; Ge, Marshall; Crow, Amanda L; Pan, Calvin; Wang, Juemei; Salehi, Pezhman; Myint, Anthony; Eskin, Eleazar; Allayee, Hooman; Lusis, Aldons J; Friedman, Rick A

2016-10-13

The discovery of environmentally specific genetic effects is crucial to the understanding of complex traits, such as susceptibility to noise-induced hearing loss (NIHL). We describe the first genome-wide association study (GWAS) for NIHL in a large and well-characterized population of inbred mouse strains, known as the Hybrid Mouse Diversity Panel (HMDP). We recorded auditory brainstem response (ABR) thresholds both pre and post 2-hr exposure to 10-kHz octave band noise at 108 dB sound pressure level in 5-6-wk-old female mice from the HMDP (4-5 mice/strain). From the observation that NIHL susceptibility varied among the strains, we performed a GWAS with correction for population structure and mapped a locus on chromosome 6 that was statistically significantly associated with two adjacent frequencies. We then used a "genetical genomics" approach that included the analysis of cochlear eQTLs to identify candidate genes within the GWAS QTL. In order to validate the gene-by-environment interaction, we compared the effects of the postnoise exposure locus with that from the same unexposed strains. The most significant SNP at chromosome 6 (rs37517079) was associated with noise susceptibility, but was not significant at the same frequencies in our unexposed study. These findings demonstrate that the genetic architecture of NIHL is distinct from that of unexposed hearing levels and provide strong evidence for gene-by-environment interactions in NIHL. Copyright © 2016 Lavinsky et al.

Hearing regulates Drosophila aggression

PubMed Central

Versteven, Marijke; Vanden Broeck, Lies; Geurten, Bart; Zwarts, Liesbeth; Decraecker, Lisse; Beelen, Melissa; Göpfert, Martin C.; Heinrich, Ralf; Callaerts, Patrick

2017-01-01

Aggression is a universal social behavior important for the acquisition of food, mates, territory, and social status. Aggression in Drosophila is context-dependent and can thus be expected to involve inputs from multiple sensory modalities. Here, we use mechanical disruption and genetic approaches in Drosophila melanogaster to identify hearing as an important sensory modality in the context of intermale aggressive behavior. We demonstrate that neuronal silencing and targeted knockdown of hearing genes in the fly’s auditory organ elicit abnormal aggression. Further, we show that exposure to courtship or aggression song has opposite effects on aggression. Our data define the importance of hearing in the control of Drosophila intermale aggression and open perspectives to decipher how hearing and other sensory modalities are integrated at the neural circuit level. PMID:28115690

[Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia].

PubMed

Dzhemileva, L U; Posukh, O L; Tazetdinov, A M; Barashkov, N A; Zhuravskiĭ, S G; Ponidelko, S N; Markova, T G; Tadinova, V N; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

2009-07-01

Mitochondrial DNA (mtDNA) mutations play an important role in etiology of hereditary hearing loss. In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. Mutations that had been shown as pathogenetically important for hearing function disturbance were identified in mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes while pathogenic role of several DNA sequences requires additional studies. This work presents the results of studying the spectrum of mutations and polymorphic variations in mtDNA genes 12S rRNA and tRNA(Ser(UGN)) in 410 patients with nonsyndromal sensoneural hearing impairment/loss from the Volga Ural region, St Petersburg, Yakutia, and Altai and in 520 individuals with normal hearing, which represent several ethnic groups (Russians, Tatars, Bashkirs, Yakuts, Altaians) residing in the Russian Federation. Pathogenetically significant mutation A1555G (12S rRNA) was found in two families (from Yakutia and St Peresburg) with hearing loss, probably caused by treatment with aminoglucosides, and in the population sample of Yakuts with a frequency of 0.83%. Further research is needed to confirm the role in hearing impairment of mutations 961insC, 961insC(n), 961delTinsC(n), T961G, T1095C (12S rRNA) and G7444A, A7445C (tRNA(Ser(UGN revealed in the patients. In addition, in the patients and the population groups, polymorphic mt DNA variants were detected, which are characteristic also of other Eurasian populations both in spectrum and frequency.

[Investigation into the relationship between mitochondrial 12 S rRNA gene, tRNA gene and cytochrome oxidase Ⅱ gene variations and the risk of noise-induced hearing loss].

PubMed

Jiao, J; Gu, G Z; Chen, G S; Li, Y H; Zhang, H L; Yang, Q Y; Xu, X R; Zhou, W H; Wu, H; He, L H; Zheng, Y X; Yu, S F

2017-01-06

Objective: To explore the relationship between mitochondrial 12 S rRNA gene variation, tRNA gene variation and cytochrome oxidase Ⅱ gene point mutations and the risk of noise-induced hearing loss (NIHL). Methods: A nested case-control study was performed that followed a cohort of 7 445 noise-exposed workers in a steel factory in Henan province, China, from January 1, 2006 to December 31, 2015. Subjects whose average hearing threshold was more than 40 dB(A) in high frequency were defined as the case group, and subjects whose average hearing threshold was less than 35 dB(A) in high frequency and less than 25 dB (A) in speech frequency were defined as the control group. Subjects was recruited into the case group ( n =286) and the control group ( n= 286) according to gender, age, job category and time of exposure to noise, and a 1∶1 case-control study was carried out. We genotyped eight single nucleotide polymorphisms in the mitochondrial 12 S rRNA gene, the mitochondrial tRNA gene and the mitochondrial cytochrome oxidase Ⅱ gene using SNPscan high-throughput genotyping technology from the recruited subjects. The relationship between polymorphic sites and NIHL, adjusted for covariates, was analyzed using conditional logistic regression analysis, as were the subgroup data. Results: The average age of the recruited subjects was (40.3±8.1) years and the length of service exposure to noise was (18.6±8.9) years. The range of noise exposed levels and cumulative noise exposure (CNE) was 80.1- 93.4 dB (A) and 86.8- 107.9 dB (A) · year, respectively. For workers exposed to noise at a CNE level<98 dB (A) · year, smokers showed an increased risk of NIHL of 1.88 (1.16-3.05) compared with non-smokers; for workers exposed to noise at a CNE level ≥98 dB(A) · year, smokers showed an increased risk of NIHL of 2.53 (1.49- 4.30) compared with non-smokers. For workers exposed to noise at a CNE level<98 dB (A) · year, the results of univariate analysis and multifactor analysis

Targeted Mutation of the Gene for Cellular Glutathione Peroxidase (Gpx1) Increases Noise-Induced Hearing Loss in Mice

PubMed Central

McFadden, Sandra L.; Ding, Da-Lian; Lear, Patricia M.; Ho, Ye-Shih

2000-01-01

Reactive oxygen species (ROS) and oxidative stress have been implicated in cochlear injury following loud noise and ototoxins. Genetic mutations that impair antioxidant defenses would be expected to increase cochlear injury following acute insults and to contribute to cumulative injury that presents as age-related hearing loss. We examined whether genetically based deficiency of cellular glutathione peroxidase, a major antioxidant enzyme, increases noise-induced hearing loss in mice. Two-month-old "knockout" mice with a targeted inactivating mutation of the gene coding for glutathione peroxidase (Gpx1) and wild type controls were exposed to broadband noise for one hour at 110 dB SPL. Auditory brainstem response (ABR) thresholds at test frequencies ranging from 5 to 40 kHz were obtained two and four weeks after exposure to determine the stable permanent component of the hearing loss. Depending on test frequency, Gpx1 knockout mice showed up to 16 dB higher ABR thresholds prior to noise exposure, and up to 15 dB greater noise-induced hearing loss, compared with controls. Within the cochlear base, there was also a significant contribution of the knockout to inner and outer hair cell loss, as well as nerve fiber loss. Our results support a link between genetic impairment of antioxidant defenses, vulnerability of the cochlea injury, and cochlear degeneration. Such impairment produces characteristics expected of some mutations associated with age-related hearing loss and offers one possible mechanism for their action. PMID:11545230

Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

PubMed Central

Pillion, Joseph P.; Vernick, David; Shapiro, Jay

2011-01-01

Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed. PMID:22567374

Utility of genetic testing for the detection of late-onset hearing loss in neonates.

PubMed

Lim, B Gail; Clark, Reese H; Kelleher, Amy S; Lin, Zhili; Spitzer, Alan R

2013-12-01

The purpose of this study was to demonstrate the utility of molecular testing in the detection of potentially important causes of delayed hearing loss missed by current audiometric screening at birth. We enrolled infants who had received a newborn audiometric hearing screen and a filter paper blood collection for state newborn screening. A central laboratory ran the SoundGene® panel. Of 3,681 infants studied, 35 (0.95%) had a positive SoundGene panel, 16 had mitochondrial mutations, 9 had Pendred mutations, 5 were cytomegalovirus (CMV) DNA positive, 2 had connexin mutations, and 3 had a combination of different mutations. Infants with an abnormal SoundGene panel were at increased risk for hearing loss compared to neonates without mutations. Three (8.6%) of the 35 subjects had persistent hearing loss compared to 5 (0.21%) of 2,398 subjects with no report of mutation (p < .01). Of 3,681 infants studied, 8 (0.22%) had persistent hearing loss: 5 (62.5%) had abnormal newborn audiometric screens, 2 (25%) had an abnormal SoundGene panel (1 was CMV positive, 1 had a mitochondrial mutation), and 1 (12.5%) had no identifiable risk factors. A positive SoundGene panel identifies infants who are not identified by audiometric testing and may be at risk for hearing loss.

Development of in vivo gene therapy for hearing disorders: introduction of adeno-associated virus into the cochlea of the guinea pig.

PubMed

Lalwani, A K; Walsh, B J; Reilly, P G; Muzyczka, N; Mhatre, A N

1996-07-01

Gene therapy is currently being used to treat many disorders including cancer, viral infection and the degenerative and fatal diseases of the cardiovascular and the central nervous systems. However, the potential use of gene therapy for alleviation of hearing impairment has not been investigated despite the absence of effective therapy for most forms of inherited hearing disorders. The purpose of this study was to assess the feasibility of introducing genetic material directly into the peripheral auditory system using adeno-associated virus (AAV) as the transfection vector and Hartley guinea pigs as the animal model. Approximately 10(5) particles of AAV containing the bacterial beta-galactosidase (beta-gal) sequence with Ad 2 major late promoter were infused into the cochlea of the animal with the aid of an osmotic minipump. Animals were killed after 2 weeks. Two Hartley guinea pigs with intracochlear saline infusion and four unoperated (nonperfused) animals served as negative controls. Both, the infused and the contralateral, non-infused cochleae were harvested from each animal, decalcified, and embedded in paraffin. Sections, 8 microns in width, were cut from the embedded cochleae and assayed for beta-gal expression via immunohistochemistry. Animals perfused with AAV showed intense immunohistochemical reactivity in the spiral limbus, spiral ligament, spiral ganglion cells and the organ of Corti in the perfused cochlea and a much weaker staining but with similar pattern in the contralateral ear. Cochleae from saline-infused and unoperated animals were devoid of the DAB stain. This study demonstrates for the first time in vivo expression of a foreign gene within the mammalian inner ear resulting from its localized, AAV-mediated introduction. The ability to introduce and stably express exogenous genetic material in the peripheral auditory system will have both experimental and therapeutic benefits. These results lay the groundwork for future studies assessing the

Autophagy is essential for hearing in mice.

PubMed

Fujimoto, Chisato; Iwasaki, Shinichi; Urata, Shinji; Morishita, Hideaki; Sakamaki, Yuriko; Fujioka, Masato; Kondo, Kenji; Mizushima, Noboru; Yamasoba, Tatsuya

2017-05-11

Hearing loss is the most frequent sensory disorder in humans. Auditory hair cells (HCs) are postmitotic at late-embryonic differentiation and postnatal stages, and their damage is the major cause of hearing loss. There is no measurable HC regeneration in the mammalian cochlea, and the maintenance of cell function is crucial for preservation of hearing. Here we generated mice deficient in autophagy-related 5 (Atg5), a gene essential for autophagy, in the HCs to investigate the effect of basal autophagy on hearing acuity. Deletion of Atg5 resulted in HC degeneration and profound congenital hearing loss. In autophagy-deficient HCs, polyubiquitinated proteins and p62/SQSTM1, an autophagy substrate, accumulated as inclusion bodies during the first postnatal week, and these aggregates increased in number. These findings revealed that basal autophagy has an important role in maintenance of HC morphology and hearing acuity.

Vestibular function in families with inherited autosomal dominant hearing loss

PubMed Central

Street, Valerie A.; Kallman, Jeremy C.; Strombom, Paul D.; Bramhall, Naomi F.; Phillips, James O.

2008-01-01

The inner ear contains the developmentally related cochlea and peripheral vestibular labyrinth. Given the similar physiology between these two organs, hearing loss and vestibular dysfunction may be expected to occur simultaneously in individuals segregating mutations in inner ear genes. Twenty-two different genes have been discovered that when mutated lead to non-syndromic autosomal dominant hearing loss. A review of the literature indicates that families segregating mutations in 13 of these 22 genes have undergone formal clinical vestibular testing. Formal assessment revealed vestibular dysfunction in families with mutations in ten of these 13 genes. Remarkably, only families with mutations in the COCH and MYO7A genes self-report considerable vestibular challenges. Families segregating mutations in the other eight genes do not self-report significant balance problems and appear to compensate well in everyday life for vestibular deficits discovered during formal clinical vestibular assessment. An example of a family (referred to as the HL1 family) with progressive hearing loss and clinically-detected vestibular hypofunction that does not report vestibular symptoms is described in this review. Notably, one member of the HL1 family with clinically-detected vestibular hypofunction reached the summit of Mount Kilimanjaro. PMID:18776598

The Application of Next-Generation Sequencing for Mutation Detection in Autosomal-Dominant Hereditary Hearing Impairment.

PubMed

Gürtler, Nicolas; Röthlisberger, Benno; Ludin, Katja; Schlegel, Christoph; Lalwani, Anil K

2017-07-01

Identification of the causative mutation using next-generation sequencing in autosomal-dominant hereditary hearing impairment, as mutation analysis in hereditary hearing impairment by classic genetic methods, is hindered by the high heterogeneity of the disease. Two Swiss families with autosomal-dominant hereditary hearing impairment. Amplified DNA libraries for next-generation sequencing were constructed from extracted genomic DNA, derived from peripheral blood, and enriched by a custom-made sequence capture library. Validated, pooled libraries were sequenced on an Illumina MiSeq instrument, 300 cycles and paired-end sequencing. Technical data analysis was performed with SeqMonk, variant analysis with GeneTalk or VariantStudio. The detection of mutations in genes related to hearing loss by next-generation sequencing was subsequently confirmed using specific polymerase-chain-reaction and Sanger sequencing. Mutation detection in hearing-loss-related genes. The first family harbored the mutation c.5383+5delGTGA in the TECTA-gene. In the second family, a novel mutation c.2614-2625delCATGGCGCCGTG in the WFS1-gene and a second mutation TCOF1-c.1028G>A were identified. Next-generation sequencing successfully identified the causative mutation in families with autosomal-dominant hereditary hearing impairment. The results helped to clarify the pathogenic role of a known mutation and led to the detection of a novel one. NGS represents a feasible approach with great potential future in the diagnostics of hereditary hearing impairment, even in smaller labs.

[From gene to disease; genetic causes of hearing loss and visual impairment sometimes accompanied by vestibular problems (Usher syndrome)].

PubMed

Pennings, R J E; Kremer, H; Deutman, A F; Kimberling, W J; Cremers, C W R J

2002-12-07

Usher syndrome is an autosomal recessively inherited disease, characterised by sensorineural hearing loss, tapetoretinal degeneration and in some cases vestibular problems. Based on the clinical heterogeneity, the disease can be classified into three clinical types (I, II and III), which have their own genetic subtypes (Usher 1A-Usher IG, Usher 2A-Usher 2C and Usher 3). The majority of the Usher type I cases are caused by mutations in the MYO7A gene (Usher 1B) while mutations in the USH2A gene (Usher 2A) are the cause of most cases of type II. Usher syndrome type III, caused by mutations in the USH3 gene, is frequently seen only in Finland.

Genomic sequencing and analyses of HearMNPV—a new Multinucleocapsid nucleopolyhedrovirus isolated from Helicoverpa armigera

PubMed Central

2012-01-01

Background HearMNPV, a nucleopolyhedrovirus (NPV), which infects the cotton bollworm, Helicoverpa armigera, comprises multiple rod-shaped nucleocapsids in virion(as detected by electron microscopy). HearMNPV shows a different host range compared with H. armigera single-nucleocapsid NPV (HearSNPV). To better understand HearMNPV, the HearMNPV genome was sequenced and analyzed. Methods The morphology of HearMNPV was observed by electron microscope. The qPCR was used to determine the replication kinetics of HearMNPV infectious for H. armigera in vivo. A random genomic library of HearMNPV was constructed according to the “partial filling-in” method, the sequence and organization of the HearMNPV genome was analyzed and compared with sequence data from other baculoviruses. Results Real time qPCR showed that HearMNPV DNA replication included a decreasing phase, latent phase, exponential phase, and a stationary phase during infection of H. armigera. The HearMNPV genome consists of 154,196 base pairs, with a G + C content of 40.07%. 162 putative ORFs were detected in the HearMNPV genome, which represented 90.16% of the genome. The remaining 9.84% constitute four homologous regions and other non-coding regions. The gene content and gene arrangement in HearMNPV were most similar to those of Mamestra configurata NPV-B (MacoNPV-B), but was different to HearSNPV. Comparison of the genome of HearMNPV and MacoNPV-B suggested that HearMNPV has a deletion of a 5.4-kb fragment containing five ORFs. In addition, HearMNPV orf66, bro genes, and hrs are different to the corresponding parts of the MacoNPV-B genome. Conclusions HearMNPV can replicate in vivo in H. armigera and in vitro, and is a new NPV isolate distinguished from HearSNPV. HearMNPV is most closely related to MacoNPV-B, but has a distinct genomic structure, content, and organization. PMID:22913743

A novel mutation of the EYA4 gene associated with post-lingual hearing loss in a proband is co-segregating with a novel PAX3 mutation in two congenitally deaf family members.

PubMed

Cesca, Federica; Bettella, Elisa; Polli, Roberta; Cama, Elona; Scimemi, Pietro; Santarelli, Rosamaria; Murgia, Alessandra

2018-01-01

This work was aimed at establishing the molecular etiology of hearing loss in a 9-year old girl with post-lingual non-syndromic mild sensorineural hearing loss with a complex family history of clinically heterogeneous deafness. The proband's DNA was subjected to NGS analysis of a 59-targeted gene panel, with the use of the Ion Torrent PGM platform. Conventional Sanger sequencing was used for segregation analysis in all the affected relatives. The proband and all the other hearing impaired members of the family underwent a thorough clinical and audiological evaluation. A new likely pathogenic mutation in the EYA4 gene (c.1154C > T; p.Ser385Leu) was identified in the proband and in her 42-year-old father with post-lingual non-syndromic profound sensorineural hearing loss. The EYA4 mutation was also found in the proband's grandfather and uncle, both showing clinical features of Waardenburg syndrome type 1. A novel pathogenic splice-site mutation (c.321+1G > A) of the PAX3 gene was found to co-segregate with the EYA4 mutation in these two subjects. The identified novel EYA4 mutation can be considered responsible of the hearing loss observed in the proband and her father, while a dual molecular diagnosis was reached in the relatives co-segregating the EYA4 and the PAX3 mutations. In these two subjects the DFNA10 phenotype was masked by Waardenburg syndrome. The use of NGS targeted gene-panel, in combination with an extensive clinical and audiological examination led us to identify the genetic cause of the hearing loss in members of a family in which different forms of autosomal dominant deafness segregate. These results provide precise and especially important prognostic and follow-up information for the future audiologic management in the youngest affected member. Copyright © 2017 Elsevier B.V. All rights reserved.

Research on Hearing and Balance--Current and Future Developments.

ERIC Educational Resources Information Center

Snow, James B., Jr.

1997-01-01

This article reviews current research that has located disease genes causing hearing impairments, discovered the ability of sensory cells of the inner ear to regenerate, developed vaccines to prevent otitis media, developed programmable hearing aids, improved cochlear implants, and demonstrated the positive effects of physical therapy with balance…

Current Status and Prospects of Gene Therapy for the Inner Ear

PubMed Central

Huang, Aji

2011-01-01

Abstract Inner ear diseases are common and often result in hearing disability. Sensorineural hearing loss is the main cause of hearing disability. So far, no effective treatment is available although some patients may benefit from a hearing aid equipped with a hearing amplifier or from cochlear implantation. Inner ear gene therapy has become an emerging field of study for the treatment of hearing disability. Numerous new discoveries and tremendous advances have been made in inner ear gene therapy including gene vectors, routes of administration, and therapeutic genes and targets. Gene therapy may become a treatment option for inner ear diseases in the near future. In this review, we summarize the current state of inner ear gene therapy including gene vectors, delivery routes, and therapeutic genes and targets by examining and analyzing publications on inner ear gene therapy from the literature and patent documents, and identify promising patents, novel techniques, and vital research projects. We also discuss the progress and prospects of inner ear gene therapy, the advances and shortcomings, with possible solutions in this field of research. PMID:21338273

Heterogeneity of Hereditary Hearing Loss in Iran: a Comprehensive Review

PubMed Central

Beheshtian, Maryam; Babanejad, Mojgan; Azaiez, Hela; Bazazzadegan, Niloofar; Kolbe, Diana; Sloan-Heggen, Christina; Arzhangi, Sanaz; Booth, Kevin; Mohseni, Marzieh; Frees, Kathy; Hossein Azizi, Mohammad; Daneshi, Ahmad; Farhadi, Mohammad; Kahrizi, Kimia; Smith, Richard JH; Najmabadi, Hossein

2017-01-01

A significant contribution to the causes of hereditary hearing impairment comes from genetic factors. More than 120 genes and 160 loci have been identified to be involved in hearing impairment. Given that consanguine populations are more vulnerable to most inherited diseases, such as hereditary hearing loss (HHL), the genetic picture of HHL among the Iranian population, which consists of at least eight ethnic subgroups with a high rate of intermarriage, is expected to be highly heterogeneous. Using an electronic literature review through various databases such as PubMed, MEDLINE, and Scopus, we review the current picture of HHL in Iran. In this review, we present more than 39 deafness genes reported to cause non-syndromic HHL in Iran, of which the most prevalent causative genes include GJB2, SLC26A4, MYO15A, and MYO7A. In addition, we highlight some of the more common genetic causes of syndromic HHL in Iran. These results are of importance for further investigation and elucidation of the molecular basis of HHL in Iran and also for developing a national diagnostic tool tailored to the Iranian context enabling early and efficient diagnosis of hereditary hearing impairment. PMID:27743438

[Association between variations in protocadherin 15 gene and occupational noise-induced hearing loss].

PubMed

Xu, X R; Yang, Q Y; Jiao, J; Zheng, Y X; He, L H; Yu, S F; Gu, G Z; Chen, G S; Zhou, W H; Wu, H; Li, Y H; Zhang, H L; Zhang, Z R

2017-01-06

Objective: The aim of this study was to investigate whether genetic variability in the protocadherin 15 (PCDH15) gene may correspond with increased susceptibility to noise-induced hearing loss (NIHL) in a Chinese population. Methods: A nested case-control study was performed that followed a cohort of 7 445 noise-exposed workers in a steel factory of Henan province in China from January 1, 2006 to December 31, 2015. In this study, 394 cases who had an average hearing threshold of more than 40 dB (A) in high frequency were defined as the case group, and 721 controls who had an average hearing threshold of less than 35 dB (A) in high frequency and less than 25 dB (A) in speech frequency were defined as the control group. A questionnaire was completed by participants and a physical test was also conducted. SNP genotyping was performed using the SNPscan TM Kit. Multivariate unconditional logistic regression additive models were used to analyze the genotypes in different groups, and the association with NIHL. Unconditional logistic regression models were used to assess the associations between the genotypes and NIHL. Results: The average age of study participants was (40.5±8.3) years and the median number of noise-exposed working years M ( P 25 , P 75 ) was 21.1 (9.1, 27.3). The range of noise exposed levels and the levels of cumulative noise exposure (CNE) were 80.1- 98.8 dB(A) and 86.6- 111.2 dB(A), respectively. Only the distribution of the genotypes (TT/CC/CT) of rs11004085 in the PCDH15 gene showed a significant difference between the case and control groups ( P= 0.049). In the case group, the distribution was 370 (93.9%), 24 (6.1%) and 0; in the control group, the distribution was 694 (96.3%), 23 (3.2%) and 1 (0.1% ). After smoking, drinking, hypertension, height and CNE adjustment, compared with the TT genotype individuals with the CC/CT genotype had a 1.90-fold increased risk of NIHL (95% CI: 1.06- 3.40). After stratified these data by the noise exposure level

Genomic analysis of a heterogeneous Mendelian phenotype: multiple novel alleles for inherited hearing loss in the Palestinian population

PubMed Central

2006-01-01

Recessively inherited phenotypes are frequent in the Palestinian population, as the result of a historical tradition of marriages within extended kindreds, particularly in isolated villages. In order to characterise the genetics of inherited hearing loss in this population, we worked with West Bank schools for the deaf to identify children with prelingual, bilateral, severe to profound hearing loss not attributable to infection, trauma or other known environmental exposure. Of 156 families enrolled, hearing loss in 17 families (11 per cent) was due to mutations in GJB2 (connexin 26), a smaller fraction of GJB2-associated deafness than in other populations. In order to estimate how many different genes might be responsible for hearing loss in this population, we evaluated ten families for linkage to all 36 known human autosomal deafness-related genes, fully sequencing hearing-related genes at any linked sites in informative relatives. Four families harboured four novel alleles of TMPRSS3 (988ΔA = 352stop), otoancorin (1067A >T = D356V) and pendrin (716T > A = V239D and 1001G > T = 346stop). In each family, all affected individuals were homozygous for the critical mutation. Each allele was specific to one or a few families in the cohort; none were widespread. Since epidemiological tests of association of mutations with deafness were not feasible for such rare alleles, we used functional and bioinformatics approaches to evaluate their consequences. In six other families, hearing loss was not linked to any known gene, suggesting that these families harbour novel genes responsible for this phenotype. We conclude that inherited hearing loss is highly heterogeneous in this population, with most extended families acting as genetic isolates in this context. We also conclude that the same genes are responsible for hearing loss in this population as elsewhere, so that gene discovery in these families informs the genetics of hearing loss worldwide. PMID:16460646

Do Hearing Protectors Protect Hearing?

PubMed Central

Groenewold, Matthew R.; Masterson, Elizabeth A.; Themann, Christa L.; Davis, Rickie R.

2015-01-01

Background We examined the association between self-reported hearing protection use at work and incidence of hearing shifts over a 5-year period. Methods Audiometric data from 19,911 workers were analyzed. Two hearing shift measures—OSHA standard threshold shift (OSTS) and high-frequency threshold shift (HFTS)—were used to identify incident shifts in hearing between workers’ 2005 and 2009 audiograms. Adjusted odds ratios were generated using multivariable logistic regression with multi-level modeling. Results The odds ratio for hearing shift for workers who reported never versus always wearing hearing protection was nonsignificant for OSTS (OR 1.23, 95% CI 0.92–1.64) and marginally significant for HFTS (OR 1.26, 95% CI 1.00–1.59). A significant linear trend towards increased risk of HFTS with decreased use of hearing protection was observed (P = 0.02). Conclusion The study raises concern about the effectiveness of hearing protection as a substitute for noise control to prevent noise-induced hearing loss in the workplace. Am. J. Ind. Med. 57:1001–1010, 2014. Published 2014. This article is a U.S. Government work and is in the public domain in the USA. PMID:24700499

Next-generation sequencing identifies three novel missense variants in ILDR1 and MYO6 genes in an Iranian family with hearing loss with review of the literature.

PubMed

Talebi, Farah; Mardasi, Farideh Ghanbari; Asl, Javad Mohammadi; Sayahi, Masoomeh

2017-12-01

Hearing impairment is the most common sensorineural disorder and is genetically heterogeneous. Identification of the pathogenic mutations underlying hearing impairment is difficult, since causative mutations in 127 different genes have so far been reported. In this study, we performed Next-generation sequencing (NGS) in 2 individuals from a consanguineous family with hearing loss. Three novel mutations in known deafness genes were identified in the family; MYO6-p.R928C and -p.D1223N in heterozygous state and ILDR1-p.Y143C in homozygous state. Sanger sequencing confirmed co-segregation of the three mutations with deafness in the family. The identified mutation in ILDR1 gene is located in the immunoglobulin-type domain of the ILDR1 protein and the detected mutations in MY06 are located in the tail domain of the MYO6 protein. The mutations are predicted to be pathogenic by SIFT, PolyPhen and Mutation Taster. Our results suggest that either the homozygous ILDR1-p.Y143C mutation might be the pathogenic variant for ARNSHL or heterozygous MYO6- p.R928C, -p.D1223N might be involved in these patient's disorder due to compound heterozygousity. To our knowledge, this is the first ILDR1 and MYO6 mutations recognized in the southwest Iran. Our data expands the spectrum of mutations in ILDR1 and MYO6 genes. Copyright © 2017 Elsevier B.V. All rights reserved.

Mutations in Cockayne Syndrome-Associated Genes (Csa and Csb) Predispose to Cisplatin-Induced Hearing Loss in Mice

PubMed Central

Rainey, Robert N.; Ng, Sum-yan; Llamas, Juan; van der Horst, Gijsbertus T. J.

2016-01-01

sensorineural hearing loss. We show that mouse models of Cockayne syndrome, a progeroid disorder resulting from a defect in the transcription-coupled DNA repair (TCR) branch of nucleotide excision repair, are hypersensitive to cisplatin-induced hearing loss and sensory hair cell death in the organ of Corti, the mammalian auditory sensory epithelium. Our work indicates that Csa and Csb, two genes involved in TCR, are preferentially required to protect against cisplatin ototoxicity, relative to global genome repair-specific elements of nucleotide excision repair, and suggests that TCR is a major force maintaining DNA integrity in the cochlea. The Cockayne syndrome mice thus represent a model for testing the contribution of DNA repair mechanisms to cisplatin ototoxicity. PMID:27122034

Mutational screening in patients with profound sensorineural hearing loss and neurodevelopmental delay: Description of a novel m.3861A > C mitochondrial mutation in the MT-ND1 gene.

PubMed

Ammar, Marwa; Tabebi, Mouna; Sfaihi, Lamia; Alila-Fersi, Olfa; Maalej, Marwa; Felhi, Rahma; Chabchoub, Imen; Keskes, Leila; Hachicha, Mongia; Fakhfakh, Faiza; Mkaouar-Rebai, Emna

2016-06-10

Mitochondrial diseases caused by mitochondrial dysfunction are a clinically and genetically, heterogeneous group of disorders involving multiple organs, particularly tissues with high-energy demand. Hearing loss is a recognized symptom of a number of mitochondrial diseases and can result from neuronal or cochlear dysfunction. The tissue affected in this pathology is most probably the cochlear hair cells, which are essential for hearing function since they are responsible for maintaining the ionic gradients necessary for sound signal transduction. Several mitochondrial DNA mutations have been associated with hearing loss and since mitochondria are crucial for the cellular energy supply in many tissues, most of these mtDNA mutations affect several tissues and will cause syndromic hearing loss. In the present study, we described 2 patients with sensorineural hearing loss and neurodevelopmental delay in whom we tested mitochondrial genes described to be associated with syndromic hearing loss. One of these patients showed a novel heteroplasmic mitochondrial mutation m.3861A > C (W185C) which lead to a loss of stability of the ND1 protein since it created a new hydrogen bund between the unique created cystein C185 and the A182 residue. In the second patient, we detected two novel heteroplasmic variations m.12350C > A (T5N) and m.14351T > C (E108G) respectively in the MT-ND5 and the MT-ND6 genes. The TopPred II prediction for the E108G variation revealed a decrease of the hydrophobicity in the mutated MT-ND6. Copyright © 2016 Elsevier Inc. All rights reserved.

Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome

PubMed Central

Hurd, Elizabeth A.; Adams, Meredith E.; Layman, Wanda S.; Swiderski, Donald L.; Beyer, Lisa A.; Halsey, Karin E.; Benson, Jennifer M.; Gong, Tzy-Wen; Dolan, David F.; Raphael, Yehoash; Martin, Donna M.

2011-01-01

Heterozygous mutations in the gene encoding chromodomain-DNA-binding-protein 7 (CHD7) cause CHARGE syndrome, a multiple anomaly condition which includes vestibular dysfunction and hearing loss. Mice with heterozygous Chd7 mutations exhibit semicircular canal dysgenesis and abnormal inner ear neurogenesis, and are an excellent model of CHARGE syndrome. Here we characterized Chd7 expression in mature middle and inner ears, analyzed morphological features of mutant ears and tested whether Chd7 mutant mice have altered responses to noise exposure and correlated those responses to inner and middle ear structure. We found that Chd7 is highly expressed in mature inner and outer hair cells, spiral ganglion neurons, vestibular sensory epithelia and middle ear ossicles. There were no obvious defects in individual hair cell morphology by Prestin immunostaining or scanning electron microscopy, and cochlear innervation appeared normal in Chd7Gt/+ mice. Hearing thresholds by auditory brainstem response (ABR) testing were elevated at 4 and 16 kHz in Chd7Gt/+ mice, and there were reduced distortion product otoacoustic emissions (DPOAE). Exposure of Chd7Gt/+ mice to broadband noise resulted in variable degrees of hair cell loss which inversely correlated with severity of stapedial defects. The degrees of hair cell loss and threshold shifts after noise exposure were more severe in wild type mice than in mutants. Together, these data indicate that Chd7Gt/+ mice have combined conductive and sensorineural hearing loss, correlating with changes in both middle and inner ears. PMID:21875659

Novel myosin mutations for hereditary hearing loss revealed by targeted genomic capture and massively parallel sequencing

PubMed Central

Brownstein, Zippora; Abu-Rayyan, Amal; Karfunkel-Doron, Daphne; Sirigu, Serena; Davidov, Bella; Shohat, Mordechai; Frydman, Moshe; Houdusse, Anne; Kanaan, Moien; Avraham, Karen B

2014-01-01

Hereditary hearing loss is genetically heterogeneous, with a large number of genes and mutations contributing to this sensory, often monogenic, disease. This number, as well as large size, precludes comprehensive genetic diagnosis of all known deafness genes. A combination of targeted genomic capture and massively parallel sequencing (MPS), also referred to as next-generation sequencing, was applied to determine the deafness-causing genes in hearing-impaired individuals from Israeli Jewish and Palestinian Arab families. Among the mutations detected, we identified nine novel mutations in the genes encoding myosin VI, myosin VIIA and myosin XVA, doubling the number of myosin mutations in the Middle East. Myosin VI mutations were identified in this population for the first time. Modeling of the mutations provided predicted mechanisms for the damage they inflict in the molecular motors, leading to impaired function and thus deafness. The myosin mutations span all regions of these molecular motors, leading to a wide range of hearing phenotypes, reinforcing the key role of this family of proteins in auditory function. This study demonstrates that multiple mutations responsible for hearing loss can be identified in a relatively straightforward manner by targeted-gene MPS technology and concludes that this is the optimal genetic diagnostic approach for identification of mutations responsible for hearing loss. PMID:24105371

The MYC Road to Hearing Restoration

PubMed Central

Kopecky, Benjamin; Fritzsch, Bernd

2012-01-01

Current treatments for hearing loss, the most common neurosensory disorder, do not restore perfect hearing. Regeneration of lost organ of Corti hair cells through forced cell cycle re-entry of supporting cells or through manipulation of stem cells, both avenues towards a permanent cure, require a more complete understanding of normal inner ear development, specifically the balance of proliferation and differentiation required to form and to maintain hair cells. Direct successful alterations to the cell cycle result in cell death whereas regulation of upstream genes is insufficient to permanently alter cell cycle dynamics. The Myc gene family is uniquely situated to synergize upstream pathways into downstream cell cycle control. There are three Mycs that are embedded within the Myc/Max/Mad network to regulate proliferation. The function of the two ear expressed Mycs, N-Myc and L-Myc were unknown less than two years ago and their therapeutic potentials remain speculative. In this review, we discuss the roles the Mycs play in the body and what led us to choose them to be our candidate gene for inner ear therapies. We will summarize the recently published work describing the early and late effects of N-Myc and L-Myc on hair cell formation and maintenance. Lastly, we detail the translational significance of our findings and what future work must be performed to make the ultimate hearing aid: the regeneration of the organ of Corti. PMID:24710525

Membrane Composition Tunes the Outer Hair Cell Motor

NASA Astrophysics Data System (ADS)

Rajagopalan, L.; Sfondouris, J.; Oghalai, J. S.; Pereira, F. A.; Brownell, W. E.

2009-02-01

Cholesterol and docosahexaenoic acid (DHA), an ω-3 fatty acid, affect membrane mechanical properties in different ways and modulate the function of membrane proteins. We have probed the functional consequence of altering cholesterol and DHA levels in the membranes of OHCs and prestin expressing HEK cells. Large, dynamic and reversible changes in prestin-associated charge movement and OHC motor activity result from altering the concentration of membrane cholesterol. Increasing membrane cholesterol shifts the q/V function ~ 50 mV in the hyperpolarizing direction, possibly a response related to increases in membrane stiffness. The voltage shift is linearly related to total membrane cholesterol. Increasing cholesterol also decreases the total charge moved in a linear fashion. Decreasing membrane cholesterol shifts the q/V function ~ 50 mV in the depolarizing direction with little or no effect on the amount of charge moved. In vivo increases in membrane cholesterol transiently increase but ultimately lead to decreases in DPOAE. Docosahexaenoic acid shifts the q/V function in the hyperpolarizing direction < 15 mV and increases total charge moved. Tuning of cochlear function by membrane cholesterol contributes to the exquisite temporal and frequency processing of mammalian hearing by optimizing the cochlear amplifier.

[Hearing loss and idoneity--the segnalation of noise-induced hearing loss hearing Loss].

PubMed

Albera, Roberto; Dagna, Federico; Cassandro, Claudia; Canale, Andrea

2011-01-01

Work idoneity in hearing loss must be related to working ability and evolution risks. Working ability is referred to the difficulties found in speech comprehension and in signals perception. As regards hearing loss evolution it is necessary to define if the subject is affected by conductive or neurosensorial hearing loss. In conductive hearing loss it is necessary to evaluate entity and frequential distribution of the deficit. In neurosensorial hearing loss it is necessary to distinguish between noise-induced hearing loss and extraprofessional hearing loss. In noise-induced hearing loss the evolution risk is high if the noise exposure is less than 10-15 years or the actual noise exposure is louder than the former. In case of extraprofessional hearing loss the evolution risk is higher in presbycusis, endolymphatic hydrops and toxic hearing loss. The necessity to report the presence on professionale noise-induced hearing loss arises if audiometric threshold is more than 25 dB at 0.5-1-2-3-4 kHz and if it is verified the professional origine of hearing loss.

Genetic basis of hearing loss in Spanish, Hispanic and Latino populations.

PubMed

Mittal, Rahul; Patel, Amit P; Nguyen, Desiree; Pan, Debbie R; Jhaveri, Vasanti M; Rudman, Jason R; Dharmaraja, Arjuna; Yan, Denise; Feng, Yong; Chapagain, Prem; Lee, David J; Blanton, Susan H; Liu, Xue Zhong

2018-03-20

Hearing loss (HL) is the most common neurosensory disorder affecting humans. The screening, prevention and treatment of HL require a better understanding of the underlying molecular mechanisms. Genetic predisposition is one of the most common factors that leads to HL. Most HL studies include few Spanish, Hispanic and Latino participants, leaving a critical gap in our understanding about the prevalence, impact, unmet health care needs, and genetic factors associated with hearing impairment among Spanish, Hispanic and Latino populations. The few studies which have been performed show that the gene variants commonly associated with HL in non-Spanish and non-Hispanic populations are infrequently responsible for hearing impairment in Spanish as well as Hispanic and Latino populations (hereafter referred to as Hispanic). To design effective screening tools to detect HL in Spanish and Hispanic populations, studies must be conducted to determine the gene variants that are most commonly associated with hearing impairment in this racial/ethnic group. In this review article, we summarize gene variants and loci associated with HL in Spanish and Hispanic populations. Identifying new genetic variants associated with HL in Spanish and Hispanic populations will pave the way to develop effective screening tools and therapeutic strategies for HL. Copyright © 2018 Elsevier B.V. All rights reserved.

The search of a genetic basis for noise-induced hearing loss (NIHL).

PubMed

Abreu-Silva, Ronaldo Serafim; Rincon, Daniel; Horimoto, Andréa Roseli Vançan Russo; Sguillar, Ary Papa; Ricardo, Luiz Artur Costa; Kimura, Lilian; Batissoco, Ana Carla; Auricchio, Maria Teresa Balester De Mello; Otto, Paulo Alberto; Mingroni-Netto, Regina Célia

2011-03-01

Knowledge about the genetic factors responsible for noise-induced hearing loss (NIHL) is still limited. This study investigated whether genetic factors are associated or not to susceptibility to NIHL. The family history and genotypes were studied for candidate genes in 107 individuals with NIHL, 44 with other causes of hearing impairment and 104 controls. Mutations frequently found among deaf individuals were investigated (35delG, 167delT in GJB2, Δ(GJB6- D13S1830), Δ(GJB6- D13S1854) in GJB6 and A1555G in MT-RNR1 genes); allelic and genotypic frequencies were also determined at the SNP rs877098 in DFNB1, of deletions of GSTM1 and GSTT1 and sequence variants in both MTRNR1 and MTTS1 genes, as well as mitochondrial haplogroups. When those with NIHL were compared with the control group, a significant increase was detected in the number of relatives affected by hearing impairment, of the genotype corresponding to the presence of both GSTM1 and GSTT1 enzymes and of cases with mitochondrial haplogroup L1. The findings suggest effects of familial history of hearing loss, of GSTT1 and GSTM1 enzymes and of mitochondrial haplogroup L1 on the risk of NIHL. This study also described novel sequence variants of MTRNR1 and MTTS1 genes.

Hearing aid fitting in older persons with hearing impairment: the influence of cognitive function, age, and hearing loss on hearing aid benefit.

PubMed

Meister, Hartmut; Rählmann, Sebastian; Walger, Martin; Margolf-Hackl, Sabine; Kießling, Jürgen

2015-01-01

To examine the association of cognitive function, age, and hearing loss with clinically assessed hearing aid benefit in older hearing-impaired persons. Hearing aid benefit was assessed using objective measures regarding speech recognition in quiet and noisy environments as well as a subjective measure reflecting everyday situations captured using a standardized questionnaire. A broad range of general cognitive functions such as attention, memory, and intelligence were determined using different neuropsychological tests. Linear regression analyses were conducted with the outcome of the neuropsychological tests as well as age and hearing loss as independent variables and the benefit measures as dependent variables. Thirty experienced older hearing aid users with typical age-related hearing impairment participated. Most of the benefit measures revealed that the participants obtained significant improvement with their hearing aids. Regression models showed a significant relationship between a fluid intelligence measure and objective hearing aid benefit. When individual hearing thresholds were considered as an additional independent variable, hearing loss was the only significant contributor to the benefit models. Lower cognitive capacity - as determined by the fluid intelligence measure - was significantly associated with greater hearing loss. Subjective benefit could not be predicted by any of the variables considered. The present study does not give evidence that hearing aid benefit is critically associated with cognitive function in experienced hearing aid users. However, it was found that lower fluid intelligence scores were related to higher hearing thresholds. Since greater hearing loss was associated with a greater objective benefit, these results strongly support the advice of using hearing aids regardless of age and cognitive function to counter hearing loss and the adverse effects of age-related hearing impairment. Still, individual cognitive capacity might

Restoration of hearing by hearing aids: conventional hearing aids – implantable hearing aids – cochlear implants – auditory brainstem implants

PubMed Central

Leuwer, R.; Müller, J.

2005-01-01

Aim of this report is to explain the current concept of hearing restoration using hearing aids. At present the main issues of conventional hearing aids are the relative benefits of analogue versus digital devices and different strategies for the improvement of hearing in noise. Implantable hearing aids provide a better sound quality and less distortion. The lack of directional microphones is the major disadvantage of the partially implantable hearing aids commercially available. Two different clinical studies about fully implantable hearing aids have been started in 2004. One of the most-promising developments seems to be the electric-acoustic stimulation. PMID:22073051

Direct Reprogramming of Spiral Ganglion Non-neuronal Cells into Neurons: Toward Ameliorating Sensorineural Hearing Loss by Gene Therapy

PubMed Central

Noda, Teppei; Meas, Steven J.; Nogami, Jumpei; Amemiya, Yutaka; Uchi, Ryutaro; Ohkawa, Yasuyuki; Nishimura, Koji; Dabdoub, Alain

2018-01-01

Primary auditory neurons (PANs) play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs), comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs). The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs. PMID:29492404

Progressive Hearing Loss in Mice Carrying a Mutation in Usp53

PubMed Central

Kazmierczak, Marcin; Harris, Suzan L.; Kazmierczak, Piotr; Shah, Prahar; Starovoytov, Valentin; Ohlemiller, Kevin K.

2015-01-01

Disordered protein ubiquitination has been linked to neurodegenerative disease, yet its role in inner ear homeostasis and hearing loss is essentially unknown. Here we show that progressive hearing loss in the ethylnitrosourea-generated mambo mouse line is caused by a mutation in Usp53, a member of the deubiquitinating enzyme family. USP53 contains a catalytically inactive ubiquitin-specific protease domain and is expressed in cochlear hair cells and a subset of supporting cells. Although hair cell differentiation is unaffected in mambo mice, outer hair cells degenerate rapidly after the first postnatal week. USP53 colocalizes and interacts with the tight junction scaffolding proteins TJP1 and TJP2 in polarized epithelial cells, suggesting that USP53 is part of the tight junction complex. The barrier properties of tight junctions of the stria vascularis appeared intact in a biotin tracer assay, but the endocochlear potential is reduced in adult mambo mice. Hair cell degeneration in mambo mice precedes endocochlear potential decline and is rescued in cochlear organotypic cultures in low potassium milieu, indicating that hair cell loss is triggered by extracellular factors. Remarkably, heterozygous mambo mice show increased susceptibility to noise injury at high frequencies. We conclude that USP53 is a novel tight junction-associated protein that is essential for the survival of auditory hair cells and normal hearing in mice, possibly by modulating the barrier properties and mechanical stability of tight junctions. SIGNIFICANCE STATEMENT Hereditary hearing loss is extremely prevalent in the human population, but many genes linked to hearing loss remain to be discovered. Forward genetics screens in mice have facilitated the identification of genes involved in sensory perception and provided valuable animal models for hearing loss in humans. This involves introducing random mutations in mice, screening the mice for hearing defects, and mapping the causative mutation

Function and expression pattern of nonsyndromic deafness genes

PubMed Central

Hilgert, Nele; Smith, Richard J.H.; Van Camp, Guy

2010-01-01

Hearing loss is the most common sensory disorder, present in 1 of every 500 newborns. To date, 46 genes have been identified that cause nonsyndromic hearing loss, making it an extremely heterogeneous trait. This review provides a comprehensive overview of the inner ear function and expression pattern of these genes. In general, they are involved in hair bundle morphogenesis, form constituents of the extracellular matrix, play a role in cochlear ion homeostasis or serve as transcription factors. During the past few years, our knowledge of genes involved in hair bundle morphogenesis has increased substantially. We give an up-to-date overview of both the nonsyndromic and Usher syndrome genes involved in this process, highlighting proteins that interact to form macromolecular complexes. For every gene, we also summarize its expression pattern and impact on hearing at the functional level. Gene-specific cochlear expression is summarized in a unique table by structure/cell type and is illustrated on a cochlear cross-section, which is available online via the Hereditary Hearing Loss Homepage. This review should provide auditory scientists the most relevant information for all identified nonsyndromic deafness genes. PMID:19601806

How to quantify binaural hearing in patients with unilateral hearing using hearing implants.

PubMed

Snik, Ad; Agterberg, Martijn; Bosman, Arjan

2015-01-01

Application of bilateral hearing devices in bilateral hearing loss and unilateral application in unilateral hearing loss (second ear with normal hearing) does not a priori lead to binaural hearing. An overview is presented on several measures of binaural benefits that have been used in patients with unilateral or bilateral deafness using one or two cochlear implants, respectively, and in patients with unilateral or bilateral conductive/mixed hearing loss using one or two percutaneous bone conduction implants (BCDs), respectively. Overall, according to this overview, the most significant and sensitive measure is the benefit in directional hearing. Measures using speech (viz. binaural summation, binaural squelch or use of the head shadow effect) showed minor benefits, except for patients with bilateral conductive/mixed hearing loss using two BCDs. Although less feasible in daily practise, the binaural masking level difference test seems to be a promising option in the assessment of binaural function. © 2015 S. Karger AG, Basel.

The physics of hearing: fluid mechanics and the active process of the inner ear.

PubMed

Reichenbach, Tobias; Hudspeth, A J

2014-07-01

Most sounds of interest consist of complex, time-dependent admixtures of tones of diverse frequencies and variable amplitudes. To detect and process these signals, the ear employs a highly nonlinear, adaptive, real-time spectral analyzer: the cochlea. Sound excites vibration of the eardrum and the three miniscule bones of the middle ear, the last of which acts as a piston to initiate oscillatory pressure changes within the liquid-filled chambers of the cochlea. The basilar membrane, an elastic band spiraling along the cochlea between two of these chambers, responds to these pressures by conducting a largely independent traveling wave for each frequency component of the input. Because the basilar membrane is graded in mass and stiffness along its length, however, each traveling wave grows in magnitude and decreases in wavelength until it peaks at a specific, frequency-dependent position: low frequencies propagate to the cochlear apex, whereas high frequencies culminate at the base. The oscillations of the basilar membrane deflect hair bundles, the mechanically sensitive organelles of the ear's sensory receptors, the hair cells. As mechanically sensitive ion channels open and close, each hair cell responds with an electrical signal that is chemically transmitted to an afferent nerve fiber and thence into the brain. In addition to transducing mechanical inputs, hair cells amplify them by two means. Channel gating endows a hair bundle with negative stiffness, an instability that interacts with the motor protein myosin-1c to produce a mechanical amplifier and oscillator. Acting through the piezoelectric membrane protein prestin, electrical responses also cause outer hair cells to elongate and shorten, thus pumping energy into the basilar membrane's movements. The two forms of motility constitute an active process that amplifies mechanical inputs, sharpens frequency discrimination, and confers a compressive nonlinearity on responsiveness. These features arise because the

The physics of hearing: fluid mechanics and the active process of the inner ear

NASA Astrophysics Data System (ADS)

Reichenbach, Tobias; Hudspeth, A. J.

2014-07-01

Most sounds of interest consist of complex, time-dependent admixtures of tones of diverse frequencies and variable amplitudes. To detect and process these signals, the ear employs a highly nonlinear, adaptive, real-time spectral analyzer: the cochlea. Sound excites vibration of the eardrum and the three miniscule bones of the middle ear, the last of which acts as a piston to initiate oscillatory pressure changes within the liquid-filled chambers of the cochlea. The basilar membrane, an elastic band spiraling along the cochlea between two of these chambers, responds to these pressures by conducting a largely independent traveling wave for each frequency component of the input. Because the basilar membrane is graded in mass and stiffness along its length, however, each traveling wave grows in magnitude and decreases in wavelength until it peaks at a specific, frequency-dependent position: low frequencies propagate to the cochlear apex, whereas high frequencies culminate at the base. The oscillations of the basilar membrane deflect hair bundles, the mechanically sensitive organelles of the ear's sensory receptors, the hair cells. As mechanically sensitive ion channels open and close, each hair cell responds with an electrical signal that is chemically transmitted to an afferent nerve fiber and thence into the brain. In addition to transducing mechanical inputs, hair cells amplify them by two means. Channel gating endows a hair bundle with negative stiffness, an instability that interacts with the motor protein myosin-1c to produce a mechanical amplifier and oscillator. Acting through the piezoelectric membrane protein prestin, electrical responses also cause outer hair cells to elongate and shorten, thus pumping energy into the basilar membrane's movements. The two forms of motility constitute an active process that amplifies mechanical inputs, sharpens frequency discrimination, and confers a compressive nonlinearity on responsiveness. These features arise because the

A new mutation in the COL4A3 gene responsible for autosomal dominant Alport syndrome, which only generates hearing loss in some carriers.

PubMed

Rosado, Consolación; Bueno, Elena; Fraile, Pilar; García-Cosmes, Pedro; González-Sarmiento, Rogelio

2015-01-01

Bilateral sensorineural hearing loss is a characteristic feature of Alport syndrome, which is always linked to renal manifestations so they have a parallel evolution and prognosis, and deafness helps to identify the renal disease. We report a family that suffers an autosomal dominant Alport syndrome caused by a previously undescribed mutation in the COL4A3 gene, in which several members have hearing impairment as the only clinical manifestation, suggesting that in this family deafness can occur independent of renal disease. This mutation is also present in a patient with anterior lenticonus, an observation only found in families with recessive and sex-linked Alport disease. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Genetics of Hearing and Deafness

PubMed Central

ANGELI, SIMON; LIN, XI; LIU, XUE ZHONG

2015-01-01

This article is a review of the genes and genetic disorders that affect hearing in humans and a few selected mouse models of deafness. Genetics is playing an increasingly critical role in the practice of medicine. This is not only in part to the importance that genetic knowledge has on traditional genetic diseases but also in part to the fact that genetic knowledge provides an understanding of the fundamental biological process of most diseases. The proteins coded by the genes related to hearing loss (HL) are involved in many functions in the ear, such as cochlear fluid homeostasis, ionic channels, stereocilia morphology and function, synaptic transmission, gene regulation, and others. Mouse models play a crucial role in understanding of the pathogenesis associated with these genes. Different types of familial HL have been recognized for years; however, in the last two decades, there has been tremendous progress in the discovery of gene mutations that cause deafness. Most of the cases of genetic deafness recognized today are monogenic disorders that can be broadly classified by the mode of inheritance (i.e., autosomal dominant, autosomal recessive, X-linked, and mitochondrial inheritance) and by the presence of associated phenotypic features (i.e., syndromic; and nonsyndromic). In terms of nonsyndromic HL, the chromosomal locations are currently known for ~ 125 loci (54 for dominant and 71 for recessive deafness), 64 genes have been identified (24 for dominant and 40 for recessive deafness), and there are many more loci for syndromic deafness and X-linked and mitochondrial DNA disorders (http://hereditaryhearingloss.org). Thus, today’s clinician must understand the science of medical genetics as this knowledge can lead to more effective disease diagnosis, counseling, treatment, and prevention. PMID:23044516

Usher syndrome: hearing loss, retinal degeneration and associated abnormalities

PubMed Central

Mathur, Pranav; Yang, Jun

2014-01-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. PMID:25481835

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.

PubMed

Mathur, Pranav; Yang, Jun

2015-03-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. Copyright © 2014 Elsevier B.V. All rights reserved.

Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome.

PubMed

Hurd, Elizabeth A; Adams, Meredith E; Layman, Wanda S; Swiderski, Donald L; Beyer, Lisa A; Halsey, Karin E; Benson, Jennifer M; Gong, Tzy-Wen; Dolan, David F; Raphael, Yehoash; Martin, Donna M

2011-12-01

Heterozygous mutations in the gene encoding chromodomain-DNA-binding-protein 7 (CHD7) cause CHARGE syndrome, a multiple anomaly condition which includes vestibular dysfunction and hearing loss. Mice with heterozygous Chd7 mutations exhibit semicircular canal dysgenesis and abnormal inner ear neurogenesis, and are an excellent model of CHARGE syndrome. Here we characterized Chd7 expression in mature middle and inner ears, analyzed morphological features of mutant ears and tested whether Chd7 mutant mice have altered responses to noise exposure and correlated those responses to inner and middle ear structure. We found that Chd7 is highly expressed in mature inner and outer hair cells, spiral ganglion neurons, vestibular sensory epithelia and middle ear ossicles. There were no obvious defects in individual hair cell morphology by prestin immunostaining or scanning electron microscopy, and cochlear innervation appeared normal in Chd7(Gt)(/+) mice. Hearing thresholds by auditory brainstem response (ABR) testing were elevated at 4 and 16 kHz in Chd7(Gt)(/+) mice, and there were reduced distortion product otoacoustic emissions (DPOAE). Exposure of Chd7(Gt)(/+) mice to broadband noise resulted in variable degrees of hair cell loss which inversely correlated with severity of stapedial defects. The degrees of hair cell loss and threshold shifts after noise exposure were more severe in wild type mice than in mutants. Together, these data indicate that Chd7(Gt)(/+) mice have combined conductive and sensorineural hearing loss, correlating with changes in both middle and inner ears. Copyright © 2011 Elsevier B.V. All rights reserved.

Hearing aid and hearing assistance technology use in Aotearoa/New Zealand.

PubMed

Kelly-Campbell, Rebecca J; Lessoway, Kamea

2015-05-01

The purpose of this study was to describe factors that are related to hearing aid and hearing assistance technology ownership and use in Aotearoa/New Zealand. Adults with hearing impairment living in New Zealand were surveyed regarding health-related quality of life and device usage. Audiometric data (hearing sensitivity and speech in noise) were collected. Data were obtained from 123 adults with hearing impairment: 73 reported current hearing-aid use, 81 reported current hearing assistance technology use. In both analyses, device users had more difficulty understanding speech in background noise, had poor hearing in both their better and worse hearing ears, and perceived more consequences of hearing impairment in their everyday lives (both emotionally and socially) than non-hearing-aid users. Discriminant analyses showed that the social consequences of hearing impairment and the better ear hearing best classified hearing aid users from non-users but social consequences and worse ear hearing best classified hearing assistance technology users from non-users. Quality of life measurements and speech-in-noise assessments provide useful clinical information. Hearing-impaired adults in New Zealand who use hearing aids also tend to use hearing assistance technology, which has important clinical implications.

Saethre-Chotzen syndrome, Pro136His TWIST mutation, hearing loss, and external and middle ear structural anomalies: report on a Brazilian family.

PubMed

Lamônica, Dionísia A C; Maximino, Luciana P; Feniman, Mariza Ribeiro; Silva, Greyce K; Zanchetta, Sthella; Abramides, Dagma V M; Passos-Bueno, Maria Rita; Rocha, Kátia; Richieri-Costa, Antonio

2010-09-01

To describe the clinical, speech, hearing, and imaging findings in three members of a Brazilian family with Saethre-Chotzen syndrome (SCS) who presented some unusual characteristics within the spectrum of the syndrome. Clinical evaluation was performed by a multidisciplinary team. Direct sequencing of the polymerase chain reaction-amplified coding region of the TWIST1 gene, routine and electrophysiological hearing evaluation, speech evaluation, and imaging studies through computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed. TWIST1 gene analysis revealed a Pro136His mutation in all patients. Hearing evaluation showed peripherial and mixed hearing loss in two of the patients, one of them with severe unilateral microtia. Computed tomography scan showed structural middle ear anomalies, and MRI showed distortion of the skull contour as well as some of the brain structures. We report a previously undescribed TWIST1 gene mutation in patients with SCS. There is evidence that indicates hearing loss (conductive and mixed) can be related both with middle ear (microtia, high jugular bulb, and enlarged vestibules) as well as with brain stem anomalies. Here we discuss the relationship between the gene mutation and the clinical, imaging, speech, and hearing findings.

Hearing Impairment

MedlinePlus

... deafness ) or loses hearing later in life (after learning to hear and speak, which is known as ... Impaired? For people who lose their hearing after learning to speak and hear, it can be difficult ...

Assessment of hearing and hearing disorders in rock/jazz musicians.

PubMed

Kähärit, Kim; Zachau, Gunilla; Eklöf, Mats; Sandsjö, Leif; Möller, Claes

2003-07-01

The aim of this study was to assess hearing and hearing disorders among rock/jazz musicians. One hundred and thirty-nine (43 women and 96 men) musicians participated. The results are based on pure-tone audiometry and questionnaire responses. According to our definition of hearing loss, tinnitus, hyperacusis, distortion and/or diplacusis as hearing disorders, we found disorders in 74%, of the rock/jazz musicians studied. Hearing loss, tinnitus and hyperacusis were most common, and the latter two were found significantly more frequently than in different reference populations. The women showed bilateral, significantly better hearing thresholds at 3-6 kHz than the men. Hyperacusis, and the combination of both hyperacusis and tinnitus, were found to be significantly more frequent among women than among men. Hearing loss and tinnitus were significantly more common among men than among women. It is important to evaluate all kinds of hearing problems (other than hearing loss) in musicians, since they represent an occupational group especially dependent on optimal, functional hearing. On the basis of our results, we suggest that hearing problems such as tinnitus, hyperacusis, distortion and/or diplacusis should, in addition to hearing loss, be defined as hearing disorders.

Association of Gel-Forming Mucins and Aquaporin Gene Expression With Hearing Loss, Effusion Viscosity, and Inflammation in Otitis Media With Effusion.

PubMed

Samuels, Tina L; Yan, Justin C; Khampang, Pawjai; Dettmar, Peter W; MacKinnon, Alexander; Hong, Wenzhou; Johnston, Nikki; Papsin, Blake C; Chun, Robert H; McCormick, Michael E; Kerschner, Joseph E

2017-08-01

Persistent, viscous middle ear effusion in pediatric otitis media (OM) contributes to increased likelihood of anesthesia and surgery, conductive hearing loss, and subsequent developmental delays. Biomarkers of effusion viscosity and hearing loss have not yet been identified despite the potential that such markers hold for targeted therapy and screening. To investigate the association of gel-forming mucins and aquaporin 5 (AQP5) gene expression with inflammation, effusion viscosity, and hearing loss in pediatric OM with effusion (OME). Case-control study of 31 pediatric patients (aged 6 months to 12 years) with OME undergoing tympanostomy tube placement and control individuals (aged 1 to 10 years) undergoing surgery for cochlear implantation from February 1, 2013, through November 30, 2014. Those with 1 or more episodes of OM in the previous 12 months, immunologic abnormality, anatomical or physiologic ear defect, OM-associated syndrome (ie, Down syndrome, cleft palate), chronic mastoiditis, or history of cholesteatoma were excluded from the study. All patients with OME and 1 control were recruited from Children's Hospital of Wisconsin, Milwaukee. The remainder of the controls were recruited from Sick Kids Hospital in Toronto, Ontario, Canada. Two to 3 middle ear biopsy specimens, effusions, and preoperative audiometric data (obtained <3 weeks before surgery) were collected from patients; only biopsy specimens were collected from controls. Expression of the mucin 2 (MUC2), mucin 5AC (MUC5AC), mucin 5B (MUC5B), and AQP5 genes were assayed in middle ear biopsy specimens by quantitative polymerase chain reaction. One middle ear biopsy specimen was sectioned for histopathologic analysis. Reduced specific viscosity of effusions was assayed using rheometry. Of the 31 study participants, 24 patients had OME (mean [SD] age, 50.4 [31.9] months; 15 [62.5%] male; 16 [66.7%] white) and 7 acted as controls (mean [SD] age, 32.6 [24.4] months; 2 [26.6%] male; 6 [85.7%] white

Pan-Canadian REspiratory STandards INitiative for Electronic Health Records (PRESTINE): 2011 national forum proceedings.

PubMed

Lougheed, M Diane; Minard, Janice; Dworkin, Shari; Juurlink, Mary-Ann; Temple, Walley J; To, Teresa; Koehn, Marc; Van Dam, Anne; Boulet, Louis-Philippe

2012-01-01

stakeholders across the spectrum of respiratory care, including clinicians, researchers, health informaticists and administrators to explore and recommend a potential scope, approach and governance structure for this important project. The Pan-Canadian REspiratory STandards INitiative for Electronic Health Records (PRESTINE) goal is to recommend respiratory data elements and standards for use in electronic medical records across Canada that meet the needs of providers, administrators, researchers and policy makers to facilitate evidence-based clinical care, monitoring, surveillance, benchmarking and policy development. The focus initially is expected to include asthma, chronic obstructive pulmonary disease and pulmonary function standards elements that are applicable to many respiratory conditions. The present article summarizes the process and findings of the forum deliberations.

Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome

PubMed Central

Chang, Qing; Wang, Jianjun; Li, Qi; Kim, Yeunjung; Zhou, Binfei; Wang, Yunfeng; Li, Huawei; Lin, Xi

2015-01-01

Mutations in the potassium channel subunit KCNQ1 cause the human severe congenital deafness Jervell and Lange-Nielsen (JLN) syndrome. We applied a gene therapy approach in a mouse model of JLN syndrome (Kcnq1−/− mice) to prevent the development of deafness in the adult stage. A modified adeno-associated virus construct carrying a Kcnq1 expression cassette was injected postnatally (P0–P2) into the endolymph, which resulted in Kcnq1 expression in most cochlear marginal cells where native Kcnq1 is exclusively expressed. We also found that extensive ectopic virally mediated Kcnq1 transgene expression did not affect normal cochlear functions. Examination of cochlear morphology showed that the collapse of the Reissner’s membrane and degeneration of hair cells (HCs) and cells in the spiral ganglia were corrected in Kcnq1−/− mice. Electrophysiological tests showed normal endocochlear potential in treated ears. In addition, auditory brainstem responses showed significant hearing preservation in the injected ears, ranging from 20 dB improvement to complete correction of the deafness phenotype. Our results demonstrate the first successful gene therapy treatment for gene defects specifically affecting the function of the stria vascularis, which is a major site affected by genetic mutations in inherited hearing loss. PMID:26084842

Acoustic trauma triggers upregulation of serotonin receptor genes

PubMed Central

Smith, Adam R.; Kwon, Jae Hyun; Navarro, Marco; Hurley, Laura M.

2014-01-01

Hearing loss induces plasticity in excitatory and inhibitory neurotransmitter systems in auditory brain regions. Excitatory-inhibitory balance is also influenced by a range of neuromodulatory regulatory systems, but less is known about the effects of auditory damage on these networks. In this work, we studied the effects of acoustic trauma on neuromodulatory plasticity in the auditory midbrain of CBA/J mice. Quantitative PCR was used to measure the expression of serotonergic and GABAergic receptor genes in the inferior colliculus (IC) of mice that were unmanipulated, sham controls with no hearing loss, and experimental individuals with hearing loss induced by exposure to a 116 dB, 10 kHz pure tone for 3 hours. Acoustic trauma induced substantial hearing loss that was accompanied by selective upregulation of two serotonin receptor genes in the IC. The Htr1B receptor gene was upregulated tenfold following trauma relative to shams, while the Htr1A gene was upregulated threefold. In contrast, no plasticity in serotonin receptor gene expression was found in the hippocampus, a region also innervated by serotonergic projections. Analyses in the IC demonstrated that acoustic trauma also changed the coexpression of genes in relation to each other, leading to an overexpression of Htr1B compared to other genes.. These data suggest that acoustic trauma induces serotonergic plasticity in the auditory system, and that this plasticity may involve comodulation of functionally-linked receptor genes. PMID:24997228

2q24 deletion in a 9-month old girl with anal atresia, hearing impairment, and hypotonia.

PubMed

Zhao, Peiwei; Mao, Bing; Cai, Xiaonan; Jiang, Jun; Liu, Zhisheng; Lin, Jun; He, Xuelian

2018-06-01

Deletion of 2q24.2 is a rare cytogenetic aberration in patients, exhibiting heterogeneous clinical features, and common phenotypes included developmental delay, intellectual disability, hypotonia, and mild dysmorphic features. Hearing impairment and anal atresia are rarely described. Here we described a 9-month-old female patient with hypotonia in all four limbs, developmental delay, and intellectual disability. In addition, congenital anal atresia was diagnosed and treated after birth, and hearing impairment was found in right ear. Single nucleotide polymorphisms (SNP) array detected a 5.2 Mb deletion on 2q24.2q24.3, including 19 genes (ITGB6; TBR1; SLC4A10; KCNH7 SCN3A; SCN2A et al.). Among these genes, it is affirmative that TBR1 is a causative gene for intellectual disability; however, the pathogenic genes of other phenotypes remain unclear. We briefly review the knowledge of genes likely involved in these clinical features, including hearing impairment, anal atresia, and developmental delay. Copyright © 2018 Elsevier B.V. All rights reserved.

Genetics of Hearing Loss – Syndromic

PubMed Central

Koffler, Tal; Ushakov, Kathy; Avraham, Karen B.

2015-01-01

Synopsis Hearing loss (HL) is one of the most common birth defects in developed countries and is a diverse pathology with different classifications. One of these is based on the association with other clinical features, defined as syndromic hearing loss (SHL). Determining the etiology of the HL in these patients is extremely beneficial as it enables a personalized approach to caring for the individual. Early screening can further aid in optimal rehabilitation for a child’s development and growth. Pathogenic variants in forty-five genes, encoding proteins functioning as ion channels, transcription factors, molecular motors and more, are known to lead to eleven forms of SHL. The development of high-throughput sequencing technology is facilitating rapid and low-cost diagnostics for patients with SHL. PMID:26443487

TAUROURSODEOXYCHOLIC ACID PREVENTS HEARING LOSS AND HAIR CELL DEATH IN Cdh23erl/erl MICE

PubMed Central

HU, J.; XU, M.; YUAN, J.; LI, B.; Entenman, S.; YU, H.; ZHENG, Q.Y.

2016-01-01

Sensorineural hearing loss has long been the subject of experimental and clinical research for many years. The recently identified novel mutation of the Cdh23 gene, Cdh23erl/erl, was proven to be a mouse model of human autosomal recessive nonsyndromic deafness (DFNB12). Tauroursodeoxycholic acid (TUDCA), a taurine-conjugated bile acid, has been used in experimental research and clinical applications related to liver disease, diabetes, neurodegenerative diseases, and other diseases associated with apoptosis. Because hair cell apoptosis was implied to be the cellular mechanism leading to hearing loss in Cdh23erl/erl mice (erl mice), this study investigated TUDCA’s otoprotective effects in erl mice: preventing hearing impairment and protecting against hair cell death. Our results showed that systemic treatment with TUDCA significantly alleviated hearing loss and suppressed hair cell death in erl mice. Additionally, TUDCA inhibited apoptotic genes and caspase-3 activation in erl mouse cochleae. The data suggest that TUDCA could be a potential therapeutic agent for human DFNB12. PMID:26748055

Hearing Screening

ERIC Educational Resources Information Center

Johnson-Curiskis, Nanette

2012-01-01

Hearing levels are threatened by modern life--headsets for music, rock concerts, traffic noises, etc. It is crucial we know our hearing levels so that we can draw attention to potential problems. This exercise requires that students receive a hearing screening for their benefit as well as for making the connection of hearing to listening.

Alternative path to hearing: photonic sonogram hearing aid

NASA Astrophysics Data System (ADS)

Hara, Elmer H.

2002-05-01

For those with total hearing loss, there are no direct remedies except for electronic (i.e. cochlear) implants. They are invasive and do not always function in a satisfactory manner. Although sign language opens the window to a rich culture but communication with the hearing world is hindered. Lip reading can bridge that gap but communication is not without some stress. Inability to detect possible life threatening situations outside the visual field also affects the quality of life for those without the ability to hear. If the hearing process is viewed from the point of system engineering, there is a sound source and air is the transmission medium to the ear. The hearing structure of the ear converts mechanical vibrations to electrical signals that are then transmitted through nerve paths to the section of the brain where sound signals are processed. In most cases of total hearing loss, the hearing structure of the ear is non-functional. A cochlear implant bypasses this hearing structure. It electronically converts sounds from the air into their frequency components and feeds them into transmission nerve paths to the brain as electrical signals. This system-engineering point of view suggests that other pathways to the brain might be explored. The following section considers the visual pathway.

Does hearing aid use affect audiovisual integration in mild hearing impairment?

PubMed

Gieseler, Anja; Tahden, Maike A S; Thiel, Christiane M; Colonius, Hans

2018-04-01

There is converging evidence for altered audiovisual integration abilities in hearing-impaired individuals and those with profound hearing loss who are provided with cochlear implants, compared to normal-hearing adults. Still, little is known on the effects of hearing aid use on audiovisual integration in mild hearing loss, although this constitutes one of the most prevalent conditions in the elderly and, yet, often remains untreated in its early stages. This study investigated differences in the strength of audiovisual integration between elderly hearing aid users and those with the same degree of mild hearing loss who were not using hearing aids, the non-users, by measuring their susceptibility to the sound-induced flash illusion. We also explored the corresponding window of integration by varying the stimulus onset asynchronies. To examine general group differences that are not attributable to specific hearing aid settings but rather reflect overall changes associated with habitual hearing aid use, the group of hearing aid users was tested unaided while individually controlling for audibility. We found greater audiovisual integration together with a wider window of integration in hearing aid users compared to their age-matched untreated peers. Signal detection analyses indicate that a change in perceptual sensitivity as well as in bias may underlie the observed effects. Our results and comparisons with other studies in normal-hearing older adults suggest that both mild hearing impairment and hearing aid use seem to affect audiovisual integration, possibly in the sense that hearing aid use may reverse the effects of hearing loss on audiovisual integration. We suggest that these findings may be particularly important for auditory rehabilitation and call for a longitudinal study.

45 CFR 81.51 - Notice of hearing or opportunity for hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 45 Public Welfare 1 2010-10-01 2010-10-01 false Notice of hearing or opportunity for hearing. 81.51 Section 81.51 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION PRACTICE AND PROCEDURE FOR HEARINGS UNDER PART 80 OF THIS TITLE Proceedings Prior to Hearing § 81.51 Notice of hearing or opportunity for hearing....

Industrial hearing conservation.

PubMed

Glorig, A

1979-08-01

Hearing conservation programs, when appropriate in industry, are now a necessity. Even though one may not wish to adopt an altruistic attitude toward the conservation of hearing, one must, like it or not, initiate a hearing conservation program because of both federal and state regulations. Since industrial noise exposure produced more hearing loss in more people than all other causes of hearing loss combined, it is incumbent on all industries with noise makers to do something about protecting human hearing. The tragedy is that nearly all industrial hearing loss can be prevented with proper hearing conservation measures. The cost of hearing conservation is far less than the cost of hearing loss in terms of human suffering and dollars in the compensation courts. Proper education of both managment and labor can result in successful hearing conservation programs. The method of choice is reduction of the noise at the source, but in many cases this is infeasible both technically and economically and therefore protection at the ear must be used. Experience has shown that with proper supervision ear protection programs can prevent the majority of instances of hearing loss in the majority of individuals exposed.

Assistive hearing technologies among students with hearing impairment: factors that promote satisfaction.

PubMed

Rekkedal, Ann Mette

2012-01-01

Hearing technology can play an essential part in the education of deaf and hard-of-hearing children in inclusive schools. Few studies have examined these children's experiences with this technology. This article explores factors pertaining to children's use of and attitudes toward hearing technologies, such as hearing aids, cochlear implants, teacher-worn microphones, and student-worn microphones. The study included 153 deaf and hard-of-hearing students. All students communicated orally and were in inclusive schools from grades 5-10. The results suggest that males view hearing technology more positively than do females. Having severe hearing loss also promoted positive attitudes toward hearing aids and cochlear implants, but not toward microphones. The students with positive self-descriptions tended to be more satisfied with hearing aids or cochlear implants than the students with negative self-descriptions. The main factors promoting the use of hearing aids were severe hearing loss, positive attitudes toward hearing aids, and the sound quality of hearing aids.

Hearing Aids and Hearing Impaired Students in Rural Schools.

ERIC Educational Resources Information Center

Woodford, Charles

This paper describes functions of the components of hearing aids and provides a detailed procedure to detect hearing aid dysfunctions. The most common type of hearing aids for school children are the behind the ear type. Various hearing aid components change sound into an electrical signal, which is amplified and adjusted by a volume control. The…

Hearing Aid Assembly

NASA Technical Reports Server (NTRS)

Grugel, Richard N. (Inventor)

2002-01-01

Progress in hearing aids has come a long way. Yet despite such progress hearing aids are not the perfect answer to many hearing problems. Some adult ears cannot accommodate tightly fitting hearing aids. Mouth movements such as chewing, talking, and athletic or other active endeavors also lead to loosely fitting ear molds. It is well accepted that loosely fitting hearing aids are the cause of feedback noise. Since feedback noise is the most common complaint of hearing aid wearers it has been the subject of various patents. Herein a hearing aid assembly is provided eliminating feedback noise. The assembly includes the combination of a hearing aid with a headset developed to constrict feedback noise.

Hearing preservation in the resection of vestibular schwannomas: patterns of hearing preservation and patient-assessed hearing function.

PubMed

Wind, Joshua J; Leonetti, John P; Raffin, Michael J M; Pisansky, Marc T; Herr, Brian; Triemstra, Justin D; Anderson, Douglas E

2011-05-01

No extant literature documents the analysis of patient perceptions of hearing as a corollary to objective audiometric measures in patients with vestibular schwannoma (VS), or acoustic neuroma. Therefore, using objective audiometric data and patient perceptions of hearing function as outlined on a questionnaire, the authors evaluated the hearing of patients who underwent VS resection. This investigation involved a retrospective review of 176 patients who had undergone VS resections in which hearing preservation was a goal. Both pre- and postoperative audiometry, expressed as a speech discrimination score (SDS) and pure tone threshold average (PTA), were performed, and the results were analyzed. Intraoperative auditory brainstem responses were also recorded. Eighty-seven of the patients (49.4%) completed a postoperative questionnaire designed to assess hearing function in a variety of social and auditory situations. Multiple linear regression analyses were completed to compare available audiometric results with questionnaire responses for each patient. One hundred forty-two patients (80.7%) had PTA and SDS audiometric data pertaining to the surgically treated ear; 94 of these patients (66.2%) had measurable postoperative hearing, as defined by a PTA < 120 dB or SD > 0%. Eighty-seven patients (49.4%) completed the retrospective questionnaire, and 74 of them had complete audiometric data and thus were included in a comparative analysis. Questionnaire data showed major postoperative subjective hearing decrements, even among patients with the same pre- and postoperative objective audiometric hearing status. Moreover, the subscore reflecting hearing while exposed to background noise, or the "cocktail party effect," characterized the most significant patient-perceived hearing deficit following VS resection. The authors' analysis of a patient-perceived hearing questionnaire showed that hearing during exposure to background noise, or the cocktail party effect, represents a

Gene mapping of the Usher syndromes.

PubMed

Kimberling, W; Smith, R J

1992-10-01

USH is an autosomal recessive group of diseases characterized by auditory impairment and visual loss owing to RP. Two common types of USH are known, types I and II. USH type I is characterized by a congenital severe to profound hearing impairment, absent vestibular function, and a progressive pigmentary retinopathy. Persons with type I do not find hearing aids useful, have delayed motor development, and experience progressive night blindness and peripheral visual loss, which usually begins in their second decade. USH type II is characterized by a congenital moderate to severe hearing loss with a down-sloping audiogram, normal vestibular function, and a progressive pigmentary retinopathy. Persons with USH2 find hearing aids beneficial, have normal psychomotor development, and experience progressive night blindness and peripheral visual loss, which usually begins in their third decade. Vestibular dysfunction is the best distinguishing hallmark to differentiate USH type I from type II. One USH type II gene (called USH2) has been assigned to chromosome 1q. One USH type I gene has been tentatively assigned to chromosome 14q. There are other USH genes that have not yet been localized.

Working Memory, Sleep, and Hearing Problems in Patients with Tinnitus and Hearing Loss Fitted with Hearing Aids.

PubMed

Zarenoe, Reza; Hällgren, Mathias; Andersson, Gerhard; Ledin, Torbjörn

2017-02-01

Tinnitus is a common condition and there is a need to evaluate effects of tinnitus management in relation to moderating factors such as degree of hearing loss. As it is possible that tinnitus influences concentration, and thus is likely to disturb cognitive processing, the role of cognitive functioning also needs to be investigated. To compare a group of patients with sensorineural hearing loss and tinnitus to a control group with only sensorineural hearing loss (and no tinnitus). To investigate working memory, sleep, and hearing problems measured before and after hearing rehabilitation. A prospective study. The sample consisted of 100 patients, 50 with hearing loss and tinnitus, and 50 controls with hearing loss but no tinnitus. All patients were between 40 and 82 yr old and had a pure-tone average (PTA; average of 0.5, 1, 2, and 4 kHz) <70 dB HL. Patients were tested before and after rehabilitation with hearing aids with regard to their working memory capacity, sleep quality, hearing problems, speech recognition, and tinnitus annoyance. Eight patients dropped out of the study. Thus, a total of 92 patients were included for analysis, with 46 in each group. As a consequence of unplanned age and PTA differences between the groups, an age-matched subsample (n = 30 + 30) was selected for further analysis. Tests including the Reading Span, Hearing-in-Noise Test (HINT), Tinnitus Handicap Inventory (THI), Hearing Handicap Inventory for the Elderly (HHIE), and Pittsburgh Sleep Quality Index (PSQI) were administered before and after hearing aid rehabilitation. There were no between-group differences at baseline in the full sample (n = 92), with the exception of the THI (p < 0.001) and the PSQI (p < 0.002), on which the hearing loss and tinnitus group had significantly higher scores. Pre/post changes were significant for both groups on the Reading Span, and HHIE. However, these improvements were significantly larger for the patients in the hearing loss and tinnitus group on

Evaluation of Extended-Wear Hearing Technology for Children with Hearing Loss.

PubMed

Wolfe, Jace; Schafer, Erin; Martella, Natalie; Morais, Mila; Mann, Misty

2015-01-01

Research shows that many older children and teenagers who have mild to moderately severe sensorineural hearing loss do not use their hearing instruments during all waking hours. A variety of reasons may contribute toward this problem, including concerns about cosmetics associated with hearing aid use and the inconvenience of daily maintenance associated with hearing instruments. Extended-wear hearing instruments are inserted into the wearer's ear canal by an audiologist and are essentially invisible to outside observers. The goal of this study was to evaluate the potential benefits and limitations associated with use of extended-wear hearing instruments in a group of children with hearing loss. A two-way repeated measures design was used to examine performance differences obtained with the participants' daily-wear hearing instruments versus that obtained with extended-wear hearing instruments. Sixteen children, ages 10-17 yr old, with sensorineural hearing loss ranging from mild to moderately severe. Probe microphone measures were completed to evaluate the aided output of device. Behavioral test measures included word recognition in quiet, sentence recognition in noise, aided warble-tone thresholds, and psychophysical loudness scaling. Questionnaires were also administered to evaluate subjective performance with each hearing technology. Data logging suggested that many participants were not using their daily-wear hearing instruments during all waking hours (mean use was less than 6 h/day). Real ear probe microphone measurements indicated that a closer fit to the Desired Sensation Level Version 5 prescriptive targets was achieved with the children's daily-wear instruments when compared to the extended-wear instruments. There was no statistically significant difference in monosyllabic word recognition at 50 or 60 dBA obtained with the two hearing technologies. Sentence recognition in noise obtained with use of the extended-wear devices was, however, significantly

Acoustic input and efferent activity regulate the expression of molecules involved in cochlear micromechanics

PubMed Central

Lamas, Veronica; Arévalo, Juan C.; Juiz, José M.; Merchán, Miguel A.

2015-01-01

Electromotile activity in auditory outer hair cells (OHCs) is essential for sound amplification. It relies on the highly specialized membrane motor protein prestin, and its interactions with the cytoskeleton. It is believed that the expression of prestin and related molecules involved in OHC electromotility may be dynamically regulated by signals from the acoustic environment. However little is known about the nature of such signals and how they affect the expression of molecules involved in electromotility in OHCs. We show evidence that prestin oligomerization is regulated, both at short and relatively long term, by acoustic input and descending efferent activity originating in the cortex, likely acting in concert. Unilateral removal of the middle ear ossicular chain reduces levels of trimeric prestin, particularly in the cochlea from the side of the lesion, whereas monomeric and dimeric forms are maintained or even increased in particular in the contralateral side, as shown in Western blots. Unilateral removal of the auditory cortex (AC), which likely causes an imbalance in descending efferent activity on the cochlea, also reduces levels of trimeric and tetrameric forms of prestin in the side ipsilateral to the lesion, whereas in the contralateral side prestin remains unaffected, or even increased in the case of trimeric and tetrameric forms. As far as efferent inputs are concerned, unilateral ablation of the AC up-regulates the expression of α10 nicotinic Ach receptor (nAChR) transcripts in the cochlea, as shown by RT-Quantitative real-time PCR (qPCR). This suggests that homeostatic synaptic scaling mechanisms may be involved in dynamically regulating OHC electromotility by medial olivocochlear efferents. Limited, unbalanced efferent activity after unilateral AC removal, also affects prestin and β-actin mRNA levels. These findings support that the concerted action of acoustic and efferent inputs to the cochlea is needed to regulate the expression of major

Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome.

PubMed

Chang, Qing; Wang, Jianjun; Li, Qi; Kim, Yeunjung; Zhou, Binfei; Wang, Yunfeng; Li, Huawei; Lin, Xi

2015-08-01

Mutations in the potassium channel subunit KCNQ1 cause the human severe congenital deafness Jervell and Lange-Nielsen (JLN) syndrome. We applied a gene therapy approach in a mouse model of JLN syndrome (Kcnq1(-/-) mice) to prevent the development of deafness in the adult stage. A modified adeno-associated virus construct carrying a Kcnq1 expression cassette was injected postnatally (P0-P2) into the endolymph, which resulted in Kcnq1 expression in most cochlear marginal cells where native Kcnq1 is exclusively expressed. We also found that extensive ectopic virally mediated Kcnq1 transgene expression did not affect normal cochlear functions. Examination of cochlear morphology showed that the collapse of the Reissner's membrane and degeneration of hair cells (HCs) and cells in the spiral ganglia were corrected in Kcnq1(-/-) mice. Electrophysiological tests showed normal endocochlear potential in treated ears. In addition, auditory brainstem responses showed significant hearing preservation in the injected ears, ranging from 20 dB improvement to complete correction of the deafness phenotype. Our results demonstrate the first successful gene therapy treatment for gene defects specifically affecting the function of the stria vascularis, which is a major site affected by genetic mutations in inherited hearing loss. © 2015 The Authors. Published under the terms of the CC BY 4.0 license.

Hearing Loss

MedlinePlus

... loud noise is a regular part of the working environment, such as farming, construction or factory work, can ... hearing tested. Consider regular hearing tests if you work in a noisy environment. Regular testing of your hearing can provide early ...

Hearing rehabilitation in Treacher Collins Syndrome with bone anchored hearing aid

PubMed Central

Polanski, José Fernando; Plawiak, Anna Clara; Ribas, Angela

2015-01-01

Objective: To describe a case of hearing rehabilitation with bone anchored hearing aid in a patient with Treacher Collins syndrome. Case description: 3 years old patient, male, with Treacher Collins syndrome and severe complications due to the syndrome, mostly related to the upper airway and hearing. He had bilateral atresia of external auditory canals, and malformation of the pinna. The initial hearing rehabilitation was with bone vibration arch, but there was poor acceptance due the discomfort caused by skull compression. It was prescribed a model of bone-anchored hearing aid, in soft band format. The results were evaluated through behavioral hearing tests and questionnaires Meaningful Use of Speech Scale (MUSS) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS). Comments: The patient had a higher acceptance of the bone-anchored hearing aid compared to the traditional bone vibration arch. Audiological tests and the speech and auditory skills assessments also showed better communication and hearing outcomes. The bone-anchored hearing aid is a good option in hearing rehabilitation in this syndrome. PMID:26298651

Touch and Emotional Availability in Hearing and Deaf or Hard of Hearing Toddlers and Their Hearing Mothers.

ERIC Educational Resources Information Center

Pipp-Siegel, Sandra; Blair, Natalie L.; Deas, Ann M.; Pressman, Leah J.; Yoshinaga-Itano, Christine

1999-01-01

A study involving 48 hearing mothers and their 2-year-old children with and without hearing impairments found mothers with children with hearing impairments touched each other more and that, unlike hearing dyads, the presence of material hostility was related to a decrease in the number of maternal and child touches. (Contains references.)…

Evaluation of family history of permanent hearing loss in childhood as a risk indicator in universal screening.

PubMed

Valido Quintana, Mercedes; Oviedo Santos, Ángeles; Borkoski Barreiro, Silvia; Santana Rodríguez, Alfredo; Ramos Macías, Ángel

Sixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation. This was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response. A total of 26,717 children were born. Eight hundred and fifty-seven (3.2%) had family history. Fifty-seven(0.21%) failed to pass the second test. A percentage of 29.1 (n=16) had another risk factor, and 17.8% (n=9) had no classical risk factor. No risk factor was related to the hearing loss except heart disease. Seventy-six point four percent had normal hearing and 23.6% hearing loss. The mean of family members with hearing loss was 1.25. On genetic testing, 82.86% of homozygotes was normal, 11.43% heterozygosity in Connexin 26 gene (35delG), 2.86% R143W heterozygosity in the same gene and 2.86% mutant homozygotes (35delG). We found no relationship between hearing loss and mutated allele. The percentage of children with a family history and hearing loss is higher than expected in the general population. The genetic profile requires updating to clarify the relationship between hearing loss and heart disease, family history and the low prevalence in the mutations analyzed. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Pan-Canadian REspiratory STandards INitiative for Electronic Health Records (PRESTINE): 2011 National Forum Proceedings

PubMed Central

Lougheed, M Diane; Minard, Janice; Dworkin, Shari; Juurlink, Mary-Ann; Temple, Walley J; To, Teresa; Koehn, Marc; Van Dam, Anne; Boulet, Louis-Philippe

2012-01-01

stakeholders across the spectrum of respiratory care, including clinicians, researchers, health informaticists and administrators to explore and recommend a potential scope, approach and governance structure for this important project. The Pan-Canadian REspiratory STandards INitiative for Electronic Health Records (PRESTINE) goal is to recommend respiratory data elements and standards for use in electronic medical records across Canada that meet the needs of providers, administrators, researchers and policy makers to facilitate evidence-based clinical care, monitoring, surveillance, benchmarking and policy development. The focus initially is expected to include asthma, chronic obstructive pulmonary disease and pulmonary function standards elements that are applicable to many respiratory conditions. The present article summarizes the process and findings of the forum deliberations. PMID:22536581

20 CFR 416.1415 - Disability hearing-disability hearing officers.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Disability hearing-disability hearing officers. 416.1415 Section 416.1415 Employees' Benefits SOCIAL SECURITY ADMINISTRATION SUPPLEMENTAL... Reopening of Determinations and Decisions Reconsideration § 416.1415 Disability hearing—disability hearing...

Hearing Loss in Adults.

ERIC Educational Resources Information Center

House, John W.

1997-01-01

This article discusses hearing loss in adults. It begins with an explanation of the anatomy of the ear and then explains the three types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed conductive-sensorineural hearing loss. Tinnitus, hearing aids, and cochlear implants are also addressed. (CR)

20 CFR 404.915 - Disability hearing-disability hearing officers.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Disability hearing-disability hearing officers. 404.915 Section 404.915 Employees' Benefits SOCIAL SECURITY ADMINISTRATION FEDERAL OLD-AGE... Determinations and Decisions Reconsideration § 404.915 Disability hearing—disability hearing officers. (a...

Congenital hearing loss

PubMed Central

Korver, Anna M. H.; Smith, Richard J. H.; Van Camp, Guy; Schleiss, Mark R.; Bitner-Glindzicz, Maria A. K.; Lustig, Lawrence R.; Usami, Shin-ichi; Boudewyns, An N.

2017-01-01

Congenital hearing loss (hearing loss present at birth) is one of the most prevalent chronic conditions in children. In the majority of developed countries, neonatal hearing-screening programmes enable early detection; early intervention will prevent delays in speech and language development and have long-lasting beneficial effects on social and emotional development and quality of life. A hearing loss diagnosis is usually followed by a search for an underlying aetiology. Congenital hearing loss might be attributed to environmental and prenatal factors, which prevail in low-income settings; congenital infections, particularly cytomegalovirus, are also a common risk factor for hearing loss. Genetic causes probably account for the majority of cases in developed countries; mutations can affect any component of the hearing pathway, in particular inner ear homeostasis (endolymph production and maintenance) and mechano-electrical transduction (conversion of a mechanical stimulus into electrochemical activity). Once the underlying cause of hearing loss is established, it might direct therapeutic decision-making and guide prevention and (genetic) counseling. Management options include specific antimicrobial therapies, surgical treatment of cranio-facial abnormalities and hearing aids. An improved understanding of the pathophysiology and molecular mechanisms underlying hearing loss and increased awareness of recent advances in genetic testing will promote the development of new treatment and screening strategies. PMID:28079113

Early onset hearing loss in autosomal recessive hypophosphatemic rickets caused by loss of function mutation in ENPP1.

PubMed

Steichen-Gersdorf, Elisabeth; Lorenz-Depiereux, Bettina; Strom, Tim Matthias; Shaw, Nicholas J

2015-07-01

Autosomal recessive hypophosphatemic rickets 2 (ARHR2) is a rare form of renal tubular phosphate wasting disorder. Loss of function mutations of the ecto-nucleotide pyrophosphatase/pyrophosphodiesterase 1 gene (ENPP1) causes a wide spectrum of phenotypes, ranging from lethal generalized arterial calcification of infancy to hypophosphatemic rickets with hypertension. Hearing loss was not previously thought to be one of the features of the disease entities and was merely regarded as a complication rather than a part of the disease. We report two children who presented in mid to late childhood with progressive varus deformity of their legs due to hypophosphatemic rickets caused by mutations in the ENPP1 gene. Both children had evidence of progressive hearing loss requiring the use of hearing aids. This report of two unrelated infants with compound heterozygous mutations in ENPP1 and previously published cases confirms that mild to moderate hearing loss is frequently associated with ARHR2. Early onset conductive hearing loss may further distinguish the autosomal recessive ENPP1 related type from other types of hypophosphatemia.

Hearing in young adults. Part I: The effects of attitudes and beliefs toward noise, hearing loss, and hearing protector devices.

PubMed

Keppler, Hannah; Dhooge, Ingeborg; Vinck, Bart

2015-01-01

There is great concern regarding the development of noise-induced hearing loss (NIHL) in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs). The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults' hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years). The questionnaire contained the Youth Attitude to Noise Scale (YANS) and Beliefs about Hearing Protection and Hearing Loss (BAHPHL). A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency) pure tone audiometry (PTA), transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA). Second, a χ² test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs) for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes.

Hearing in young adults. Part I: The effects of attitudes and beliefs toward noise, hearing loss, and hearing protector devices

PubMed Central

Keppler, Hannah; Dhooge, Ingeborg; Vinck, Bart

2015-01-01

There is great concern regarding the development of noise-induced hearing loss (NIHL) in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs). The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults’ hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years). The questionnaire contained the Youth Attitude to Noise Scale (YANS) and Beliefs about Hearing Protection and Hearing Loss (BAHPHL). A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency) pure tone audiometry (PTA), transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA). Second, a χ2 test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs) for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes. PMID:26356365

A mutation in the gamma actin 1 (ACTG1) gene causes autosomal dominant hearing loss (DFNA20/26)

PubMed Central

van Wijk, E; Krieger, E; Kemperman, M; De Leenheer, E M R; Huygen, P; Cremers, C; Cremers, F; Kremer, H

2003-01-01

Linkage analysis in a multigenerational family with autosomal dominant hearing loss yielded a chromosomal localisation of the underlying genetic defect in the DFNA20/26 locus at 17q25-qter. The 6-cM critical region harboured the γ-1-actin (ACTG1) gene, which was considered an attractive candidate gene because actins are important structural elements of the inner ear hair cells. In this study, a Thr278Ile mutation was identified in helix 9 of the modelled protein structure. The alteration of residue Thr278 is predicted to have a small but significant effect on the γ 1 actin structure owing to its close proximity to a methionine residue at position 313 in helix 11. Met313 has no space in the structure to move away. Moreover, the Thr278 residue is highly conserved throughout eukaryotic evolution. Using a known actin structure the mutation could be predicted to impair actin polymerisation. These findings strongly suggest that the Thr278Ile mutation in ACTG1 represents the first disease causing germline mutation in a cytoplasmic actin isoform. PMID:14684684

Variable hearing impairment in a DFNB2 family with a novel MYO7A missense mutation.

PubMed

Hildebrand, M S; Thorne, N P; Bromhead, C J; Kahrizi, K; Webster, J A; Fattahi, Z; Bataejad, M; Kimberling, W J; Stephan, D; Najmabadi, H; Bahlo, M; Smith, R J H

2010-06-01

Myosin VIIA mutations have been associated with non-syndromic hearing loss (DFNB2; DFNA11) and Usher syndrome type 1B (USH1B). We report clinical and genetic analyses of a consanguineous Iranian family segregating autosomal recessive non-syndromic hearing loss (ARNSHL). The hearing impairment was mapped to the DFNB2 locus using Affymetrix 50K GeneChips; direct sequencing of the MYO7A gene was completed. The Iranian family (L-1419) was shown to segregate a novel homozygous missense mutation (c.1184G>A) that results in a p.R395H amino acid substitution in the motor domain of the myosin VIIA protein. As one affected family member had significantly less severe hearing loss, we used a candidate approach to search for a genetic modifier. This novel MYO7A mutation is the first reported to cause DFNB2 in the Iranian population and this DFNB2 family is the first to be associated with a potential modifier. The absence of vestibular and retinal defects, and less severe low frequency hearing loss, is consistent with the phenotype of a recently reported Pakistani DFNB2 family. Thus, we conclude this family has non-syndromic hearing loss (DFNB2) rather than USH1B, providing further evidence that these two diseases represent discrete disorders.

Treatment Efficacy: Hearing Aids in the Management of Hearing Loss in Adults.

ERIC Educational Resources Information Center

Weinstein, Barbara E.

1996-01-01

This article reviews the efficacy of hearing aids in adults with hearing impairments. Information is provided on the prevalence of hearing impairments; the daily effects of a hearing impairment; and the role of the audiologist. The effectiveness and benefits of hearing aids are reviewed, and a case study is provided. (CR)

The HEAR-QL: Quality of Life Questionnaire for Children with Hearing Loss

PubMed Central

Umansky, Amy M.; Jeffe, Donna B.; Lieu, Judith E.C.

2012-01-01

Background Few quality of life (QOL) assessment tools are available for children with specific chronic conditions, and none have been designed specifically for children with hearing loss (HL). A validated hearing-related QOL questionnaire could help clinicians determine whether an intervention is beneficial and whether one intervention is better than another. Purpose To examine QOL in children with HL and assess the validity, reliability, and factor structure of a new measure, the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire. Research Design A descriptive and correlational study of a convenience sample of children. Study Sample Participants included 35 children with unilateral HL, 45 with bilateral HL, and 35 siblings with normal hearing. Data Collection and Analysis Children 7-12 years old were recruited by mail from a tertiary-care pediatric otolaryngology practice and the local county's Special School District. With parent consent, children completed the validated Pediatric Quality of Life Inventory™ (PedsQL™) 4.0 and a 35-item HEAR-QL questionnaire. The factor structure of the HEAR-QL was determined through principal components analysis (PCA), and mean scores were computed for each subscale and the total HEAR-QL. Three weeks following return of the initial questionnaires, a second HEAR-QL questionnaire was sent to participants to assess test-retest reliability. Both PedsQL and HEAR-QL scores were compared between children with and without HL, between children with unilateral and bilateral HL, and between children who used and did not use a hearing device using analysis of variance. Sensitivity and specificity were calculated for both the HEAR-QL and PedsQL. A multivariable, hierarchical linear regression analysis was conducted with independent variables associated with HEAR-QL in unadjusted tests. Results Using exploratory PCA, the 35-item HEAR-QL was reduced to 26 items (Cronbach's α=0.97; sensitivity 91% and specificity

The HEAR-QL: quality of life questionnaire for children with hearing loss.

PubMed

Umansky, Amy M; Jeffe, Donna B; Lieu, Judith E C

2011-01-01

Few quality of life (QOL) assessment tools are available for children with specific chronic conditions, and none have been designed specifically for children with hearing loss (HL). A validated hearing-related QOL questionnaire could help clinicians determine whether an intervention is beneficial and whether one intervention is better than another. To examine QOL in children with HL and assess the validity, reliability, and factor structure of a new measure, the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire. A descriptive and correlational study of a convenience sample of children. Participants included 35 children with unilateral HL, 45 with bilateral HL, and 35 siblings with normal hearing. Children 7-12 yr old were recruited by mail from a tertiary-care pediatric otolaryngology practice and the local county's Special School District. With parent consent, children completed the validated Pediatric Quality of Life Inventory™ (PedsQL) 4.0 and a 35-item HEAR-QL questionnaire. The factor structure of the HEAR-QL was determined through principal components analysis (PCA), and mean scores were computed for each subscale and the total HEAR-QL. Three weeks following the return of the initial questionnaires, a second HEAR-QL questionnaire was sent to participants to assess test-retest reliability. Both PedsQL and HEAR-QL scores were compared between children with and without HL, between children with unilateral and bilateral HL, and between children who used and did not use a hearing device using analysis of variance. Sensitivity and specificity were calculated for both the HEAR-QL and the PedsQL. A multivariable, hierarchical linear regression analysis was conducted with independent variables associated with the HEAR-QL in unadjusted tests. Using exploratory PCA, the 35-item HEAR-QL was reduced to 26 items (Cronbach's α = 0.97, sensitivity of 91% and specificity of 92% at a cutoff score of 93.5) loading on three factors: difficulty

TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad.

PubMed

Guillen-Ahlers, Hector; Erbe, Christy B; Chevalier, Frédéric D; Montoya, Maria J; Zimmerman, Kip D; Langefeld, Carl D; Olivier, Michael; Runge, Christina L

2018-04-19

Sensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized. A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD. Whole-exome sequencing was performed to identify coding sequence variants shared by both family members, and screened against genes relevant to hearing loss and variants known to be associated with SNHL and ANSD. Both family members are successful cochlear implant users, demonstrating effective auditory nerve stimulation with their devices. Genetic analyses revealed a mutation (rs35725509) in the TMTC2 gene, which has been reported previously as a likely genetic cause of SNHL in another family of Northern European descent. This study represents the first confirmation of the rs35725509 variant in an independent family as a likely cause for the complex hearing loss phenotype (SNHL and ANSD) observed in this family dyad. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

Do You Hear Me? Information for Persons with a Hearing Impairment.

ERIC Educational Resources Information Center

Brook, Emily

1990-01-01

This compendium of general information for individuals with hearing impairments reports on: the incidence of hearing impairments; methods of communication between hearing and nonhearing persons (fingerspelling, lip reading, cued speech, signing, and telephone devices); 13 titles of books and tapes available; 4 sources of hearing ear dogs; 42…

Rurality and determinants of hearing healthcare in adult hearing aid recipients.

PubMed

Chan, Stephen; Hixon, Brian; Adkins, Margaret; Shinn, Jennifer B; Bush, Matthew L

2017-10-01

The objective of this study was to compare the timing of hearing aid (HA) acquisition between adults in rural and urban communities. We hypothesized that time of acquisition of HA after onset of hearing loss is greater in rural adults compared with urban adults. Secondary objectives included assessment of socioeconomic/educational status and impact of hearing loss and hearing rehabilitation of urban and rural HA recipients. Cross-sectional questionnaire survey. We assessed demographics, timing of HA fitting from onset of hearing loss, and impact of hearing impairment in 336 adult HA recipients (273 urban, 63 rural) from a tertiary referral center. Amplification benefit was assessed using the International Outcome Inventory for Hearing Aids (IOI). The time to HA acquisition was greater for rural participants compared to urban participants (19.1 vs. 25.7 years, P = 0.024) for those with untreated hearing loss for at least 8 years. Age at hearing loss onset was correlated with time to HA acquisition (P = -0.54, P < 0.001). Rural HA participants experienced longer commutes to hearing specialists (68 vs. 32 minutes, P < 0.001), were less likely to achieve a degree beyond high school (P < 0.001), and were more likely to possess Medicaid coverage (P = 0.012) compared to urban participants. Hearing impairment caused job performance difficulty in 60% of all participants. Rural adults are at risk for delayed HA acquisition, which may be related to distance to hearing specialists. Further research is indicated to investigate barriers to care and expand access for vulnerable populations. 4. Laryngoscope, 127:2362-2367, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Local gene transfection in the cochlea (Review).

PubMed

Xia, Li; Yin, Shankai

2013-07-01

There is much interest in the potential application of vector-induced gene therapeutic approaches to several forms of hearing disorders due to the poor efficacy of existing treatments. The cochlea is an ideal site for local gene transfection due to its anatomical encapsulation and fluid flow within its ducts. However, this requires the development of novel technologies in materials science and microbial supply vectors for target gene delivery. This review focuses on the introduction of various viral and non-viral vectors as well as injection approaches to transfecting cochlear cells in vivo. Finally, the perspective of local gene therapy was discussed. Therapeutic approaches using local gene transfection may provide a means of cochlear cell and tissue protection and treatment in cases of exogenous hearing loss and endogenous disorders.

The Master Hearing Aid

PubMed Central

Curran, James R.

2013-01-01

As early as the 1930s the term Master Hearing Aid (MHA) described a device used in the fitting of hearing aids. In their original form, the MHA was a desktop system that allowed for simulated or actual adjustment of hearing aid components that resulted in a changed hearing aid response. Over the years the MHA saw many embodiments and contributed to a number of rationales for the fitting of hearing aids. During these same years, the MHA was viewed by many as an inappropriate means of demonstrating hearing aids; the audio quality of the desktop systems was often superior to the hearing aids themselves. These opinions and the evolution of the MHA have molded the modern perception of hearing aids and the techniques used in the fitting of hearing aids. This article reports on a history of the MHA and its influence on the fitting of hearing aids. PMID:23686682

Binaural pitch fusion: Comparison of normal-hearing and hearing-impaired listenersa)

PubMed Central

Reiss, Lina A. J.; Shayman, Corey S.; Walker, Emily P.; Bennett, Keri O.; Fowler, Jennifer R.; Hartling, Curtis L.; Glickman, Bess; Lasarev, Michael R.; Oh, Yonghee

2017-01-01

Binaural pitch fusion is the fusion of dichotically presented tones that evoke different pitches between the ears. In normal-hearing (NH) listeners, the frequency range over which binaural pitch fusion occurs is usually <0.2 octaves. Recently, broad fusion ranges of 1–4 octaves were demonstrated in bimodal cochlear implant users. In the current study, it was hypothesized that hearing aid (HA) users would also exhibit broad fusion. Fusion ranges were measured in both NH and hearing-impaired (HI) listeners with hearing losses ranging from mild-moderate to severe-profound, and relationships of fusion range with demographic factors and with diplacusis were examined. Fusion ranges of NH and HI listeners averaged 0.17 ± 0.13 octaves and 1.7 ± 1.5 octaves, respectively. In HI listeners, fusion ranges were positively correlated with a principal component measure of the covarying factors of young age, early age of hearing loss onset, and long durations of hearing loss and HA use, but not with hearing threshold, amplification level, or diplacusis. In NH listeners, no correlations were observed with age, hearing threshold, or diplacusis. The association of broad fusion with early onset, long duration of hearing loss suggests a possible role of long-term experience with hearing loss and amplification in the development of broad fusion. PMID:28372056

Deaf and hearing parents' interactions with eldest hearing children.

PubMed

Jones, E G; Dumas, R E

1996-10-01

Observational methods were used to gather data from 54 individuals in 9 Deaf-parented families and 9 hearing-parented families whose eldest hearing children were aged 7 to 11 years old. Transcripts of parent/child dyadic interactions during a vacation planning activity were analyzed using Condon, Cooper and Grotevant's (1984) Individuation Code. Repeated measures ANOVAS were conducted with the independent variables of the parent's gender (mother versus fathers) and group membership (Deaf-parented versus hearing-parented families) on the percentage of communication that reflected self-assertion, separateness, permeability, and mutuality. There were no significant differences between children from Deaf-parented families versus children from hearing-parented families. There was a significant main effect for parents in communication reflecting self-assertion; namely hearing parents had a higher percentage of self-assertive communication than Deaf parents.

Satisfaction with Hearing Aids Based on Technology and Style among Hearing Impaired Persons

PubMed Central

Faraji- Khiavi, Farzad; Dashti, Rezvan; Sameni, Seyyed-Jalal; Bayat, Arash

2016-01-01

Introduction: Hearing loss is one of the most disabling impairments. Using a hearing aid as an attempt to improve the hearing problem can positively affect the quality of life for these people. This research was aimed to assess satisfaction of hearing impaired patients with their hearing aids regarding the employed technology and style. Materials and Methods: This descriptive-analytic cross-sectional research was conducted on 187 subjects with hearing loss who were using a hearing aid. The subjects were over 18 years of age and were using a hearing aid for at least 6 months. The Persian version of Satisfaction with Amplification in Daily Life (SADL) questionnaire was the instrument which was used for assessing satisfaction with the hearing aid. Cronbach’s alpha was calculated to be 0.80 for instrument reliability. Results: A significant difference was observed among satisfaction subscales’ mean scores with hearing aid technology. Also a significant difference was observed between the total satisfaction score and the hearing aid model. With respect to the analysis of satisfaction with the hearing aid and its style, cost and services was the only subscale which showed a significant difference (P=0.005). Conclusion: Respondents using hearing aids with different technology and style were estimated to be quite satisfied. Training audiologists in using more appropriate and fitting hearing aids in addition to using self-reporting questionnaires like SADL for estimating patients’ social condition and participation in their life can essentially change their disability condition and countervail their hearing loss. PMID:27738608

[Newborn hearing screening program: association between hearing loss and risk factors].

PubMed

Pereira, Priscila Karla Santana; Martins, Adriana de Souza; Vieira, Márcia Ribeiro; Azevedo, Marisa Frasson de

2007-01-01

Hearing loss in newborns. To verify the prevalence of auditory alterations in newborns of Hospital São Paulo (hospital), observing if there are any correlations with the following variables: birth weight, gestational age, relation weight/gestational age and risk factors for hearing loss. A retrospective analysis of the hospital records of 1696 newborns; 648 records of preterm infants and 1048 records of infants born at term. All of the infants had been submitted to an auditory evaluation consisting of: Transient Otoacoustic Emissions, investigation of the cochleal-palpebral reflexes and acoustic imittance tests, identifying the type and level of hearing loss. Sensorineural hearing loss was identified in .82% of the infants who were born at term and in 3.1% of the preterm infants -- with a statistically significant difference. Conductive hearing loss was the most frequent type of hearing loss in both groups, occurring in 14.6% of the term infants and in 16.3% of the preterm infants. Alteration of the central auditory system was considered as a possible diagnosis for 5.8% of the preterm infants and for 3.3% of the term infants. For the group of infants who were born at term, a significant correlation was observed between failure in the hearing screening test and the presence of risk factors such as family history and presence of a syndrome -- the child who presented a syndrome had 37 times more chances of failing in the hearing screening test and seven times more chances of failing in the right ear when there was a family history for hearing loss. The lower the gestational age (< 30 weeks) and birth weight (< 1500 g), the higher the chances of failing in the hearing screening test (3 times more). Hearing loss had a higher occurrence in preterm infants who remained in the ICU. Gestational age and birth weight were important variables related to the possibility of failure in the hearing screening test. A correlation was observed between the presence of a syndrome and

Frequency of Usher syndrome in two pediatric populations: Implications for genetic screening of deaf and hard of hearing children.

PubMed

Kimberling, William J; Hildebrand, Michael S; Shearer, A Eliot; Jensen, Maren L; Halder, Jennifer A; Trzupek, Karmen; Cohn, Edward S; Weleber, Richard G; Stone, Edwin M; Smith, Richard J H

2010-08-01

Usher syndrome is a major cause of genetic deafness and blindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed > or =1 pathogenic mutations in any Usher gene. Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Usher syndrome is more prevalent than has been reported before the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs.

Frequency of Usher Syndrome in Two Pediatric Populations: Implications for genetic screening of Deaf and Hard of Hearing Children

PubMed Central

Kimberling, William J.; Hildebrand, Michael S.; Shearer, A. Eliot; Jensen, Maren L.; Halder, Jennifer A.; Cohn, Edward S.; Weleber, Richard G.; Stone, Edwin M.; Smith, Richard J. H.

2011-01-01

Purpose Usher syndrome is a major cause of genetic deafblindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. Methods A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed ≥1 pathogenic mutations in any Usher gene. Results Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Conclusion Usher syndrome is more prevalent than has been reported prior to the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs. PMID:20613545

Perceptions Toward Internet-Based Delivery of Hearing Aids among Older Hearing-Impaired Adults.

PubMed

Chandra, Navshika; Searchfield, Grant D

2016-06-01

Despite evidence that hearing aids can improve the social and psychological functioning of older hearing-impaired adults, hearing aid uptake is low. High cost of hearing aids and poor access to audiology services in rural areas are potential barriers to hearing aid acquisition. Methods of hearing aid delivery deviating from the traditional clinician-based model have been available to consumers for many years. One such method is Internet hearing aid sales. However, research exploring Internet-based hearing aid delivery, as a method to improve hearing aid uptake in this population, is limited. The purpose of this study was to explore the perceptions of older hearing aid users (aged ≥65 yr) toward Internet-based hearing aid delivery. A qualitative approach was adopted to investigate older adults' perceptions of buying hearing aids online. The sample consisted of 18 participants aged between 64 and 81 yr. Fourteen men and four women participated in this study. Participants were all experienced hearing aid users. Face-to-face semistructured interviews were conducted. An interview schedule guided the interview. Interviews were recorded with a voice recorder and transcribed verbatim. Thematic analysis of the data was carried out. Seven main themes emerged from the data. A general lack of awareness, but willingness to learn more about Internet hearing aid sales, was found. Two perceived benefits of Internet-based hearing aid delivery were identified: lower cost of hearing aids and greater convenience or physical accessibility. Numerous concerns and limitations were communicated. Concerns regarding the availability of clinical procedures, such as hearing tests, obtaining the correct-sized earmolds, and fine-tuning of hearing aids, were expressed. Participants conveyed distrust in online retailers. However, trust in and a preference for audiologists' expertise, which was not perceived to be available online, was found. Participants further conveyed a preference for face

How to Get Hearing Aids

MedlinePlus

... batteries and hearing aids away from children and pets. We recommend visiting a hearing healthcare professional on a regular basis to have your hearing aids inspected. More in Hearing Aids Hearing Loss Types of Hearing Aids Benefits and Safety Issues Hearing Aids and Cell Phones ...

Diversity of the causal genes in hearing impaired Algerian individuals identified by whole exome sequencing

PubMed Central

Ammar-Khodja, Fatima; Bonnet, Crystel; Dahmani, Malika; Ouhab, Sofiane; Lefèvre, Gaelle M; Ibrahim, Hassina; Hardelin, Jean-Pierre; Weil, Dominique; Louha, Malek; Petit, Christine

2015-01-01

The genetic heterogeneity of congenital hearing disorders makes molecular diagnosis expensive and time-consuming using conventional techniques such as Sanger sequencing of DNA. In order to design an appropriate strategy of molecular diagnosis in the Algerian population, we explored the diversity of the involved mutations by studying 65 families affected by autosomal recessive forms of nonsyndromic hearing impairment (DFNB forms), which are the most prevalent early onset forms. We first carried out a systematic screening for mutations in GJB2 and the recurrent p.(Arg34*) mutation in TMC1, which were found in 31 (47.7%) families and 1 (1.5%) family, respectively. We then performed whole exome sequencing in nine of the remaining families, and identified the causative mutations in all the patients analyzed, either in the homozygous state (eight families) or in the compound heterozygous state (one family): (c.709C>T: p.(Arg237*)) and (c.2122C>T: p.(Arg708*)) in OTOF, (c.1334T>G: p.(Leu445Trp)) in SLC26A4, (c.764T>A: p.(Met255Lys)) in GIPC3, (c.518T>A: p.(Cys173Ser)) in LHFPL5, (c.5336T>C: p.(Leu1779Pro)) in MYO15A, (c.1807G>T: p.(Val603Phe)) in OTOA, (c.6080dup: p.(Asn2027Lys*9)) in PTPRQ, and (c.6017del: p.(Gly2006Alafs*13); c.7188_7189ins14: p.(Val2397Leufs*2)) in GPR98. Notably, 7 of these 10 mutations affecting 8 different genes had not been reported previously. These results highlight for the first time the genetic heterogeneity of the early onset forms of nonsyndromic deafness in Algerian families. PMID:26029705

Prevalence of GJB2 Mutations in Affected Individuals from United Arab Emirates with Autosomal Recessive Nonsyndromic Hearing Loss.

PubMed

Tlili, Abdelaziz; Al Mutery, Abdullah; Kamal Eddine Ahmad Mohamed, Walaa; Mahfood, Mona; Hadj Kacem, Hassen

2017-11-01

Mutations in the gap junction protein beta 2 (GJB2) gene are responsible for more cases of nonsyndromic recessive hearing loss than any other gene. The purpose of our study was to evaluate the prevalence of GJB2 mutations among affected individuals from United Arab Emirates (UAE). There were 50 individuals diagnosed with hereditary hearing loss and 120 healthy individuals enrolled in the study. The Sanger sequencing method was used to screen the GJB2 coding region in all affected individuals. The c.-1G>A variant was determined by the polymerase chain reaction-restriction fragment length polymorphism method in normal individuals. Nine cases with bi-allelic mutations and three cases with mono-allelic mutations were detected in 12 out of 50 patients (24%). The homozygous mutation c.35delG was identified as the cause of hearing loss in six participants (12%). The mutation c.506G>A was identified in three affected individuals (6%). The allelic frequency (14%) and low percentage of individuals that were homozygous (2%) for the c.35delG mutation suggest that there are other genes responsible for nonsyndromic deafness in the UAE population. The results reported here are a preliminary step in collecting epidemiological data regarding autosomal recessive nonsyndromic hearing loss related to GJB2 gene mutations among the UAE population. The c.35delG mutation of the GJB2 gene is the most frequently seen causative mutation in the UAE and is followed by the p.Cys169Tyr mutation.

Hearing loss caused by a P2RX2 mutation identified in a MELAS family with a coexisting mitochondrial 3243AG mutation

PubMed Central

Moteki, Hideaki; Azaiez, Hela; Booth, Kevin T; Hattori, Mitsuru; Sato, Ai; Sato, Yoshihiko; Motobayashi, Mitsuo; Sloan, Christina M; Kolbe, Diana L; Shearer, A Eliot; Smith, Richard J H; Usami, Shin-ichi

2015-01-01

Objective We present a family with a mitochondrial DNA 3243A>G mutation resulting in MELAS, of which some members have hearing loss where a novel mutation in the P2RX2 gene was identified. Methods One hundred ninety-four (194) Japanese subjects from unrelated families were enrolled in the study. Targeted genomic enrichment and massively parallel sequencing of all known non-syndromic hearing loss genes were performed to identify the genetic causes of hearing loss. Results A novel mutation in the P2RX2 gene, that corresponded to c.601G>A (p.Asp201Tyr) was identified. Two patients carried the mutation, and had severe SNHL, while other members with MELAS (who did not carry the P2RX2 mutation) had normal hearing. Conclusion This is the first case report of a diagnosis of hearing loss caused by P2RX2 mutation in patients with MELAS. A potential explanation is that decreasing ATP production due to MELAS with mitochondrial 3243A>G mutation might suppress activation of P2X2 receptors. We also suggest that hearing loss caused by the P2RX2 mutation might be influenced by the decrease in ATP production due to MELAS, and that nuclear genetic factors may play a modifying role in mitochondrial dysfunction. PMID:25788561

Hearing in the elderly. Relation of hearing loss, loneliness, and self-esteem.

PubMed

Chen, H L

1994-06-01

1. The prevalence of hearing impairment increases with age. Hearing handicap resulting from hearing loss may lead to psychosocial problems in elders. 2. Previous studies have shown relations among hearing handicap, loneliness and low self-esteem, but those studies focused on populations other than elders. 3. Early detection of hearing handicap and appropriate interventions promote increased independence and preserve the ability of elders to interact with their environment.

Exploring the influence of culture on hearing help-seeking and hearing-aid uptake.

PubMed

Zhao, Fei; Manchaiah, Vinaya; St Claire, Lindsay; Danermark, Berth; Jones, Lesley; Brandreth, Marian; Krishna, Rajalakshmi; Goodwin, Robin

2015-07-01

The purpose of this paper was to highlight the importance of cultural influence in understanding hearing-help seeking and hearing-aid uptake. Information on audiological services in different countries and 'theories related to cross-culture' is presented, followed by a general discussion. Twenty-seven relevant literature reviews on hearing impairment, cross-cultural studies, and the health psychology model and others as secondary resources. Despite the adverse consequences of hearing impairment and the significant potential benefits of audiological rehabilitation, only a small number of those with hearing impairment seek professional help and take up appropriate rehabilitation. Therefore, hearing help-seeking and hearing-aid uptake has recently become the hot topic for clinicians and researchers. Previous research has identified many contributing factors for hearing help-seeking with self-reported hearing disability being one of the main factors. Although significant differences in help-seeking and hearing-aid adoption rates have been reported across countries in population studies, limited literature on the influence of cross-cultural factors in this area calls for an immediate need for research. This paper highlights the importance of psychological models and cross-cultural research in the area of hearing help-seeking and hearing-aid uptake, and consequently some directions for future research are proposed.

Identification of a novel homozygous mutation in MYO3A in a Chinese family with DFNB30 non-syndromic hearing impairment.

PubMed

Qu, Ronggui; Sang, Qing; Xu, Yao; Feng, Ruizhi; Jin, Li; He, Lin; Wang, Lei

2016-05-01

Hearing loss is a common sensory impairment. Several genetic loci or genes responsible for non-syndrome hearing loss have been identified, including the well-known deafness genes GJB2, MT-RNR1 and SLC26A4. MYO3A belongs to the myosin superfamily. Previously only three mutations in this gene have been found in an Isreali family with DFNB30, in which patients demonstrated progressive hearing loss. In this study, we characterized a consanguineous Kazakh family with congenital hearing loss. By targeted sequence capture and next-generation sequencing, we identified a homozygous mutation and did bioinformatics analysis to this mutation. A homozygous mutation, MYO3A:c.1841C>T (p.S614F), was identified to be responsible for the disease. Ser614 is located in the motor domain of MYO3A that is highly conserved among different species. Molecular modeling predicts that the conserved Ser614 may play an important role in maintaining the stability of β-sheet and the interaction between neighboring β-strand. This is the second report on MYO3A mutations in deafness and the first report in China. The finding help facilitate establishing a better relationship between MYO3A mutation and hearing phenotypes. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Clinical Validity of hearScreen™ Smartphone Hearing Screening for School Children.

PubMed

Mahomed-Asmail, Faheema; Swanepoel, De Wet; Eikelboom, Robert H; Myburgh, Hermanus C; Hall, James

2016-01-01

The study aimed to determine the validity of a smartphone hearing screening technology (hearScreen™) compared with conventional screening audiometry in terms of (1) sensitivity and specificity, (2) referral rate, and (3) test time. One thousand and seventy school-age children in grades 1 to 3 (8 ± 1.1 average years) were recruited from five public schools. Children were screened twice, once using conventional audiometry and once with the smartphone hearing screening. Screening was conducted in a counterbalanced sequence, alternating initial screen between conventional or smartphone hearing screening. No statistically significant difference in performance between techniques was noted, with smartphone screening demonstrating equivalent sensitivity (75.0%) and specificity (98.5%) to conventional screening audiometry. While referral rates were lower with the smartphone screening (3.2 vs. 4.6%), it was not significantly different (p > 0.05). Smartphone screening (hearScreen™) was 12.3% faster than conventional screening. Smartphone hearing screening using the hearScreen™ application is accurate and time efficient.

Mathematics Motivation, Anxiety, and Performance in Female Deaf/Hard-of-Hearing and Hearing Students

ERIC Educational Resources Information Center

Ariapooran, Saeed

2017-01-01

Hearing loss can be a major detriment to academic achievement among students. The present comparative study examines the differences in mathematics motivation, anxiety, and performance in female students with hearing loss and their hearing peers. A total of 63 female students with hearing loss (deaf and hard-of-hearing) and 63 hearing female…

Bilateral versus unilateral hearing aids for bilateral hearing impairment in adults.

PubMed

Schilder, Anne Gm; Chong, Lee Yee; Ftouh, Saoussen; Burton, Martin J

2017-12-19

Acquired hearing loss is common and its incidence increases markedly with age. In most people, 'age-related' hearing loss is sensorineural (due to the loss of cochlear hair cells) and bilateral, affecting both ears to the same degree. Hearing loss categorised as mild, moderate or severe is primarily managed with hearing aids. People with bilateral hearing loss may be offered one aid, fitted to one specific ear, or two aids fitted to both ears. There is uncertainty about the relative benefits to people with hearing loss of these different strategies. To assess the effects of bilateral versus unilateral hearing aids in adults with a bilateral hearing impairment. The Cochrane ENT Information Specialist searched the ENT Trials Register; Cochrane Register of Studies Online; PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 8 June 2017. Randomised controlled trials (RCTs) comparing the fitting of two versus one ear-level acoustic hearing aids in adults (over 18 years) with a bilateral hearing impairment, both ears being eligible for hearing aids. We used the standard methodological procedures expected by Cochrane. Our primary outcomes were patient preference for bilateral or unilateral aids, hearing-specific health-related quality of life and adverse effects (pain or discomfort in the ear, initiation or exacerbation of middle or outer ear infection). Secondary outcomes included: usage of hearing aids (as measured by, for example, data logging or battery consumption), generic health-related quality of life, listening ability and audiometric benefit measured as binaural loudness summation. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics. We included four cross-over RCTs with a total of 209 participants, ranging in age from 23 to 85 and with a preponderance of men. All the studies allowed the use of hearing aids for

Hearing loss in space

NASA Technical Reports Server (NTRS)

Buckey, J. C. Jr; Musiek, F. E.; Kline-Schoder, R.; Clark, J. C.; Hart, S.; Havelka, J.

2001-01-01

BACKGROUND: Temporary and, in some cases, permanent hearing loss has been documented after long-duration spaceflights. METHODS: We examined all existing published data on hearing loss after space missions to characterize the losses. RESULTS: Data from Russian missions suggest that the hearing loss, when it occurs, affects mainly mid to high frequencies and that using hearing protection often might prevent the loss. Several significant questions remain about hearing loss in space. While the hearing loss has been presumed to be noise-induced, no clear link has been established between noise exposure and hearing loss during spaceflight. In one documented case of temporary hearing loss from the Shuttle-Mir program, the pattern of loss was atypical for a noise-induced loss. Continuous noise levels that have been measured on the Mir and previous space stations, while above engineering standards, are not at levels usually associated with hearing loss in ground-based studies (which have usually been limited to 8-10 h exposure periods). Attempts to measure hearing in space using threshold-based audiograms have been unsuccessful in both the American and Russian programs due to noise interference with the measurements. CONCLUSIONS: The existing data highlight the need for reliable monitoring of both hearing and noise in long-duration spaceflight.

Gene Therapy Restores Balance and Auditory Functions in a Mouse Model of Usher Syndrome.

PubMed

Isgrig, Kevin; Shteamer, Jack W; Belyantseva, Inna A; Drummond, Meghan C; Fitzgerald, Tracy S; Vijayakumar, Sarath; Jones, Sherri M; Griffith, Andrew J; Friedman, Thomas B; Cunningham, Lisa L; Chien, Wade W

2017-03-01

Dizziness and hearing loss are among the most common disabilities. Many forms of hereditary balance and hearing disorders are caused by abnormal development of stereocilia, mechanosensory organelles on the apical surface of hair cells in the inner ear. The deaf whirler mouse, a model of human Usher syndrome (manifested by hearing loss, dizziness, and blindness), has a recessive mutation in the whirlin gene, which renders hair cell stereocilia short and dysfunctional. In this study, wild-type whirlin cDNA was delivered to the inner ears of neonatal whirler mice using adeno-associated virus serotype 2/8 (AAV8-whirlin) by injection into the posterior semicircular canal. Unilateral whirlin gene therapy injection was able to restore balance function as well as improve hearing in whirler mice for at least 4 months. Our data indicate that gene therapy is likely to become a treatment option for hereditary disorders of balance and hearing. Copyright © 2017. Published by Elsevier Inc.

Childhood Hearing Health: Educating for Prevention of Hearing Loss

PubMed Central

Lacerda, Adriana Bender Moreira; Gonçalves, Claudia Giglio de Oliveira; Lacerda, Giselle; Lobato, Diolén Conceição Barros; Santos, Luciana; Moreira, Aline Carlezzo; Ribas, Angela

2014-01-01

Introduction The presence of noise in our society has attracted the attention of health professionals, including speech-language pathologists, who have been charged along with educators with developing hearing conservation programs in schools. Objective To describe the results of three strategies for awareness and hearing preservation in first to fourth grades in public elementary schools. Methods The level of environmental noise in classrooms was assessed, and 638 elementary school students from first to fourth grades, 5 to 10 years of age, were audiologically evaluated. After the evaluations, educational activities were presented to children and educators. Results The noise level in the classroom ranged from 71.8 to 94.8 A-weighted decibels. The environment of the classroom was found to promote sound reverberation, which hinders communication. Thirty-two students (5.1%) presented hearing alterations. Conclusion The application of strategies for a hearing conservation program at the school showed that noise is present in the room, and hearing loss, sometimes silent, affects schoolchildren. Students and teachers were aware that hearing problems can be prevented. Avoiding exposure to noise and improving the acoustics in classrooms are essential. PMID:25992146

Identifying congenital hearing impairment. Personal experience based on selective hearing screening.

PubMed

Molini, E; Ricci, G; Baroni, S; Ciorba, A; Bellocci, A; Simoncelli, C

2004-06-01

If all degrees of permanent uni- or bilateral hypoacusis are taken into consideration, hearing impairment is the most common congenital disease. Early detection of permanent infantile hearing impairment has become extremely important in preventive medicine, since steps can be taken with hearing aids and rehabilitation to ensure better development of language and higher cognitive functions. Aim of this study is to provide a critical review of the time of diagnosis of hypoacusis at our audiology laboratory, where two methods were used to screen hearing of children with/without risk indicators. Results of approximately 10 years' work were re-examined during which time outpatient screening was conducted on children referred by colleagues in neonatology and paediatrics. All were carriers of congenital risk indicators associated with sensorineural and/or conductive hearing loss, based on the Joint Committee on Infant Hearing findings, or were suspected of being hypoacusic even if they had no known congenital risk factors. Hearing screening was conducted in hospital on newborns with no risk factors, within the first few days of birth. Results of the present study showed that when selective hearing screening was performed, the mean age of high-risk patients diagnosed with hypoacusis was slightly higher than that in international guidelines. Moreover, these patients represent approximately half the hypoacusic population identified in the study period. The other half of congenital hypoacusic subjects identified had no risk indicators and there was a significant delay in diagnosis due to later manifestation of symptoms indicating hypoacusis, and thus, in turn, delayed referral for hearing tests. In contrast, subjects without risk indicators who underwent in-hospital hearing screening and proved to be hypoacusic, were diagnosed early. In our experience, however, universal screening has considerable disadvantages, such as difficulty in covering the entire population, difficulty

On Membrane Motor Activity and Chloride Flux in the Outer Hair Cell: Lessons Learned from the Environmental Toxin Tributyltin

PubMed Central

Song, Lei; Seeger, Achim; Santos-Sacchi, Joseph

2005-01-01

The outer hair cell (OHC) underlies mammalian cochlea amplification, and its lateral membrane motor, prestin, which drives the cell's mechanical activity, is modulated by intracellular chloride ions. We have previously described a native nonselective conductance (GmetL) that influences OHC motor activity via Cl flux across the lateral membrane. Here we further investigate this conductance and use the environmental toxin tributyltin (TBT) to better understand Cl-prestin interactions. Capitalizing on measures of prestin-derived nonlinear capacitance to gauge Cl flux across the lateral membrane, we show that the Cl ionophore TBT, which affects neither the motor nor GmetL directly, is capable of augmenting the native flux of Cl in OHCs. These observations were confirmed using the chloride-sensitive dye MQAE. Furthermore, the compound's potent ability, at nanomolar concentrations, to equilibrate intra- and extracellular Cl concentrations is shown to surpass the effectiveness of GmetL in promoting Cl flux, and secure a quantitative analysis of Cl-prestin interactions in intact OHCs. Using malate as an anion replacement, we quantify chloride effects on the nonlinear charge density and operating voltage range of prestin. Our data additionally suggest that ototoxic effects of organotins can derive from their disruption of OHC Cl homeostasis, ultimately interfering with anionic modulation of the mammalian cochlear amplifier. Notably, this observation identifies a new environmental threat for marine mammals by TBT, which is known to accumulate in the food chain. PMID:15596517

Genetics of Hearing Loss

MedlinePlus

... Hearing Loss Homepage Basics Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2016 2015 2014 2013 ...

Hearing Loss in Children

MedlinePlus

... Hearing Loss Homepage Basics Noise-Induced Hearing Loss Genetics of Hearing Loss Screening & Diagnosis Types of Hearing Loss About Sound Treatment & Intervention Services Learning Language Bacterial Meningitis Studies Data & Statistics EHDI Annual Data 2016 2015 2014 2013 ...

Exploration of a physiologically-inspired hearing-aid algorithm using a computer model mimicking impaired hearing.

PubMed

Jürgens, Tim; Clark, Nicholas R; Lecluyse, Wendy; Meddis, Ray

2016-01-01

To use a computer model of impaired hearing to explore the effects of a physiologically-inspired hearing-aid algorithm on a range of psychoacoustic measures. A computer model of a hypothetical impaired listener's hearing was constructed by adjusting parameters of a computer model of normal hearing. Absolute thresholds, estimates of compression, and frequency selectivity (summarized to a hearing profile) were assessed using this model with and without pre-processing the stimuli by a hearing-aid algorithm. The influence of different settings of the algorithm on the impaired profile was investigated. To validate the model predictions, the effect of the algorithm on hearing profiles of human impaired listeners was measured. A computer model simulating impaired hearing (total absence of basilar membrane compression) was used, and three hearing-impaired listeners participated. The hearing profiles of the model and the listeners showed substantial changes when the test stimuli were pre-processed by the hearing-aid algorithm. These changes consisted of lower absolute thresholds, steeper temporal masking curves, and sharper psychophysical tuning curves. The hearing-aid algorithm affected the impaired hearing profile of the model to approximate a normal hearing profile. Qualitatively similar results were found with the impaired listeners' hearing profiles.

Diphtheria and hearing loss.

PubMed

Schubert, C R; Cruickshanks, K J; Wiley, T L; Klein, R; Klein, B E; Tweed, T S

2001-01-01

To determine if infectious diseases usually experienced in childhood have an effect on hearing ability later in life. The Epidemiology of Hearing Loss Study (N = 3,753) is a population-based study of age-related hearing loss in adults aged 48 to 92 years in Beaver Dam, Wisconsin. As part of this study, infectious disease history was obtained and hearing was tested using pure-tone audiometry. Hearing loss was defined as a pure-tone average of thresholds at 500 Hz, 1,000 Hz, 2,000 Hz, and 4,000 Hz greater than 25 decibels hearing level in either ear. After adjusting for confounders, only a history of diphtheria (n = 37) was associated with hearing loss (odds ratio [OR] 2.79; 95% confidence interval [CI] 1.05, 7.36). There was no relationship between hearing loss and history of chickenpox, measles, mumps, pertussis, polio, rheumatic fever, rubella, or scarlet fever. Only two participants with a history of diphtheria and hearing loss reported having a hearing loss before age 20. Diphtheria in childhood may have consequences for hearing that do not become apparent until later in life. A possible biological mechanism for a diphtheria effect on hearing ability exists: The toxin produced by the Corynebacterium diphtheriae bacteria can cause damage to cranial nerves and therefore may affect the auditory neural pathway. These data may have important implications for areas facing a resurgence of diphtheria cases.

Assistive Hearing Technologies among Students with Hearing Impairment: Factors that Promote Satisfaction

ERIC Educational Resources Information Center

Rekkedal, Ann Mette

2012-01-01

Hearing technology can play an essential part in the education of deaf and hard-of-hearing children in inclusive schools. Few studies have examined these children's experiences with this technology. This article explores factors pertaining to children's use of and attitudes toward hearing technologies, such as hearing aids, cochlear implants,…

Hearing loss in Waardenburg syndrome: a systematic review.

PubMed

Song, J; Feng, Y; Acke, F R; Coucke, P; Vleminckx, K; Dhooge, I J

2015-06-22

Waardenburg syndrome (WS) is a rare genetic disorder characterized by hearing loss (HL) and pigment disturbances of hair, skin and iris. Classifications exist based on phenotype and genotype. The auditory phenotype is inconsistently reported among the different Waardenburg types and causal genes, urging the need for an up-to-date literature overview on this particular topic. We performed a systematic review in search for articles describing auditory features in WS patients along with the associated genotype. Prevalences of HL were calculated and correlated with the different types and genes of WS. Seventy-three articles were included, describing 417 individual patients. HL was found in 71.0% and was predominantly bilateral and sensorineural. Prevalence of HL among the different clinical types significantly differed (WS1: 52.3%, WS2: 91.6%, WS3: 57.1%, WS4: 83.5%). Mutations in SOX10 (96.5%), MITF (89.6%) and SNAI2 (100%) are more frequently associated with hearing impairment than other mutations. Of interest, the distinct disease-causing genes are able to better predict the auditory phenotype compared with different clinical types of WS. Consequently, it is important to confirm the clinical diagnosis of WS with molecular analysis in order to optimally inform patients about the risk of HL. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Expressivity of hearing loss in cases with Usher syndrome type IIA.

PubMed

Sadeghi, André M; Cohn, Edward S; Kimberling, William J; Halvarsson, Glenn; Möller, Claes

2013-12-01

The purpose of this study was to compare the genotype/phenotype relationship between siblings with identical USH2A pathologic mutations and the consequent audiologic phenotypes, in particular degree of hearing loss (HL). Decade audiograms were also compared among two groups of affected subjects with different mutations of USH2A. DNA samples from patients with Usher syndrome type II were analysed. The audiological features of patients and affected siblings with USH2A mutations were also examined to identify genotype-phenotype correlations. Genetic and audiometric examinations were performed in 18 subjects from nine families with Usher syndrome type IIA. Three different USH2A mutations were identified in the affected subjects. Both similarities and differences of the auditory phenotype were seen in families with several affected siblings. A variable degree of hearing loss, ranging from mild to profound, was observed among affected subjects. No significant differences in hearing thresholds were found the group of affected subjects with different pathological mutations. Our results indicate that mutations in the USH2A gene and the resulting phenotype are probably modulated by other variables, such as modifying genes, epigenetics or environmental factors which may be of importance for better understanding the etiology of Usher syndrome.

Quality of Life and Hearing Eight Years After Sudden Sensorineural Hearing Loss.

PubMed

Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Vasama, Juha-Pekka

2017-04-01

To explore long-term hearing results, quality of life (QoL), quality of hearing (QoH), work-related stress, tinnitus, and balance problems after idiopathic sudden sensorineural hearing loss (ISSNHL). Cross-sectional study. We reviewed the audiograms of 680 patients with unilateral ISSNHL on average 8 years after the hearing impairment, and then divided the patients into two study groups based on whether their ISSNHL had recovered to normal (pure tone average [PTA] ≤ 30 dB) or not (PTA > 30 dB). The inclusion criteria were a hearing threshold decrease of 30 dB or more in at least three contiguous frequencies occurring within 72 hours in the affected ear and normal hearing in the contralateral ear. Audiograms of 217 patients fulfilled the criteria. We reviewed their medical records; measured present QoL, QoH, and work-related stress with specific questionnaires; and updated the hearing status. Poor hearing outcome after ISSNHL was correlated with age, severity of hearing loss, and vertigo together with ISSNHL. Quality of life and QoH were statistically significantly better in patients with recovered hearing, and the patients had statistically significantly less tinnitus and balance problems. During the 8-year follow-up, the PTA of the affected ear deteriorated on average 7 dB, and healthy ear deteriorated 6 dB. Idiopathic sudden sensorineural hearing loss that failed to recover had a negative impact on long-term QoL and QoH. The hearing deteriorated as a function of age similarly both in the affected and the healthy ear, and there were no differences between the groups. The cumulative recurrence rate for ISSNHL was 3.5%. 4 Laryngoscope, 127:927-931, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Pragmatic Abilities of Children with Hearing Loss Using Cochlear Implants or Hearing Aids Compared to Hearing Children

ERIC Educational Resources Information Center

Most, Tova; Shina-August, Ella; Meilijson, Sara

2010-01-01

This study characterized the profile of pragmatic abilities among 24 children with hearing loss (HL) aged 6.3-9.4 years, 13 using hearing aids (HAs) and 11 using cochlear implants (CIs), in comparison to those of 13 hearing children with similar chronological and language ages. All the children with HL used spoken language, attended regular…

18 CFR 1308.33 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the original...

18 CFR 1308.33 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 18 Conservation of Power and Water Resources 2 2014-04-01 2014-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the original...

18 CFR 1308.33 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 18 Conservation of Power and Water Resources 2 2012-04-01 2012-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the original...

18 CFR 1308.33 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 18 Conservation of Power and Water Resources 2 2013-04-01 2012-04-01 true Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the original...

18 CFR 1308.33 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 1308.33... Prehearing and Hearing Procedures § 1308.33 Hearings. (a) TVA shall arrange for the verbatim reporting of evidentiary hearings before the Hearing Officer, and shall provide the Hearing Officer with the original...

What factors influence help-seeking for hearing impairment and hearing aid adoption in older adults?

PubMed

Meyer, Carly; Hickson, Louise

2012-02-01

The purpose of this review paper was to identify factors that influence older adults' decisions to seek help for hearing impairment and to adopt hearing aids. A literature search of abstracts within peer-reviewed journal articles published in English between 1990 and 2010 was conducted in online bibliographic databases using the keywords: hearing; impair* or loss; seek* or candidate* or applicant* or acquir*; and hearing aid* or help or advice. Twenty-two articles that were directly relevant to this topic were included in this review. The evidence suggests that individuals are more likely to seek help for hearing impairment and/or adopt hearing aids if they: (1) have a moderate to severe hearing impairment and self-reported hearing-related activity limitations or participation restrictions; (2) are older; (3) perceive their hearing as poor; (4) consider there to be more benefits than barriers to amplification; and (5) perceive their significant other/s as supportive of hearing rehabilitation. A barrier to help-seeking for hearing impairment and hearing aid adoption appears to be general practitioners' management of age-related hearing impairment.

Application of Mouse Models to Research in Hearing and Balance.

PubMed

Ohlemiller, Kevin K; Jones, Sherri M; Johnson, Kenneth R

2016-12-01

Laboratory mice (Mus musculus) have become the major model species for inner ear research. The major uses of mice include gene discovery, characterization, and confirmation. Every application of mice is founded on assumptions about what mice represent and how the information gained may be generalized. A host of successes support the continued use of mice to understand hearing and balance. Depending on the research question, however, some mouse models and research designs will be more appropriate than others. Here, we recount some of the history and successes of the use of mice in hearing and vestibular studies and offer guidelines to those considering how to apply mouse models.

22 CFR 401.23 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 22 Foreign Relations 2 2012-04-01 2009-04-01 true Hearings. 401.23 Section 401.23 Foreign... Hearings. (a) The time and place of the hearing or hearings of an application shall be fixed by the... of the hearing or hearings to the applicant, the Governments and all persons who have presented...

22 CFR 401.23 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 22 Foreign Relations 2 2014-04-01 2014-04-01 false Hearings. 401.23 Section 401.23 Foreign... Hearings. (a) The time and place of the hearing or hearings of an application shall be fixed by the... of the hearing or hearings to the applicant, the Governments and all persons who have presented...

13 CFR 117.16 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 13 Business Credit and Assistance 1 2012-01-01 2012-01-01 false Hearings. 117.16 Section 117.16... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 117.15... Hearings and Appeals (OHA) that the matter be scheduled for hearing; or (2) Advise the applicant or...

22 CFR 401.23 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 22 Foreign Relations 2 2013-04-01 2009-04-01 true Hearings. 401.23 Section 401.23 Foreign... Hearings. (a) The time and place of the hearing or hearings of an application shall be fixed by the... of the hearing or hearings to the applicant, the Governments and all persons who have presented...

22 CFR 512.21 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-04-01

....21 Hearing. (a) Petition for hearing. (1) A hearing may be requested by filing a written petition...) was hand delivered or the date of delivery by certified mail. (4) Where petition is received after the.... (b) Type of hearing. (1) The form and content of the hearing will be determined by the hearing...

13 CFR 117.16 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 13 Business Credit and Assistance 1 2010-01-01 2010-01-01 false Hearings. 117.16 Section 117.16... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 117.15... Hearings and Appeals (OHA) that the matter be scheduled for hearing; or (2) Advise the applicant or...

Applications of direct-to-consumer hearing devices for adults with hearing loss: a review.

PubMed

Manchaiah, Vinaya; Taylor, Brian; Dockens, Ashley L; Tran, Nicole R; Lane, Kayla; Castle, Mariana; Grover, Vibhu

2017-01-01

This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1) personal sound amplification products (PSAPs), 2) direct-mail hearing aids, and 3) over-the-counter (OTC) hearing aids. A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review. Included studies fell into three domains: 1) electroacoustic characteristics, 2) consumer surveys, and 3) outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level). Low-end (or low-cost) devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis), especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%-19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive outcomes of OTC devices in the elderly population. Of note, OTC outcomes appear better when a hearing health care professional supports these users. While some direct-to-consumer hearing devices have the capability to produce adverse effects due to production of dangerously high sound levels and internal noise, the existing literature suggests that there are potential benefits of these devices. Research of direct-to-consumer hearing devices is limited, and current published studies are of weak quality. Much effort is needed to understand the

Hearing-aid tester

NASA Technical Reports Server (NTRS)

Kessinger, R.; Polhemus, J. T.; Waring, J. G.

1977-01-01

Hearing aids are automatically checked by circuit that applies half-second test signal every thirty minutes. If hearing-aid output is distorted, too small, or if battery is too low, a warning lamp is activated. Test circuit is incorporated directly into hearing-aid package.

Cortical Auditory Evoked Potentials in (Un)aided Normal-Hearing and Hearing-Impaired Adults

PubMed Central

Van Dun, Bram; Kania, Anna; Dillon, Harvey

2016-01-01

Cortical auditory evoked potentials (CAEPs) are influenced by the characteristics of the stimulus, including level and hearing aid gain. Previous studies have measured CAEPs aided and unaided in individuals with normal hearing. There is a significant difference between providing amplification to a person with normal hearing and a person with hearing loss. This study investigated this difference and the effects of stimulus signal-to-noise ratio (SNR) and audibility on the CAEP amplitude in a population with hearing loss. Twelve normal-hearing participants and 12 participants with a hearing loss participated in this study. Three speech sounds—/m/, /g/, and /t/—were presented in the free field. Unaided stimuli were presented at 55, 65, and 75 dB sound pressure level (SPL) and aided stimuli at 55 dB SPL with three different gains in steps of 10 dB. CAEPs were recorded and their amplitudes analyzed. Stimulus SNRs and audibility were determined. No significant effect of stimulus level or hearing aid gain was found in normal hearers. Conversely, a significant effect was found in hearing-impaired individuals. Audibility of the signal, which in some cases is determined by the signal level relative to threshold and in other cases by the SNR, is the dominant factor explaining changes in CAEP amplitude. CAEPs can potentially be used to assess the effects of hearing aid gain in hearing-impaired users. PMID:27587919

22 CFR 401.29 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 22 Foreign Relations 2 2013-04-01 2009-04-01 true Hearings. 401.29 Section 401.29 Foreign... Hearings. (a) A hearing or hearings may be held whenever in the opinion of the Commission such action would... or exceptions which may be imposed by the terms of the reference, a final hearing or hearings shall...

22 CFR 401.29 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 22 Foreign Relations 2 2012-04-01 2009-04-01 true Hearings. 401.29 Section 401.29 Foreign... Hearings. (a) A hearing or hearings may be held whenever in the opinion of the Commission such action would... or exceptions which may be imposed by the terms of the reference, a final hearing or hearings shall...

20 CFR 658.417 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 20 Employees' Benefits 4 2014-04-01 2014-04-01 false Hearings. 658.417 Section 658.417 Employees... Hearings. (a) Hearings shall be held by State hearing officials. A State hearing official may be any State official authorized to hold hearings under State law. They may be, for example, the same referees who hold...

22 CFR 401.29 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 22 Foreign Relations 2 2014-04-01 2014-04-01 false Hearings. 401.29 Section 401.29 Foreign... Hearings. (a) A hearing or hearings may be held whenever in the opinion of the Commission such action would... or exceptions which may be imposed by the terms of the reference, a final hearing or hearings shall...

20 CFR 658.417 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 20 Employees' Benefits 4 2012-04-01 2012-04-01 false Hearings. 658.417 Section 658.417 Employees... Hearings. (a) Hearings shall be held by State hearing officials. A State hearing official may be any State official authorized to hold hearings under State law. They may be, for example, the same referees who hold...

Do You Hear What Horton Hears?

ERIC Educational Resources Information Center

Snyder, Robert; Johnson, Jordan

2010-01-01

"I've never heard of a small speck of dust that is able to yell" says Horton of a sound he hears well (Geisel 1954). It is always valuable to connect science to student's interests and their everyday world--so what better way to teach concepts relating to sound than to read "Horton Hears a Who" by Dr. Seuss? Here the authors present several…

Hearing

ERIC Educational Resources Information Center

Koehlinger, Keegan M.; Van Horne, Amanda J. Owen; Moeller, Mary Pat

2013-01-01

Purpose: Spoken language skills of 3- and 6-year-old children who are hard of hearing (HH) were compared with those of children with normal hearing (NH). Method: Language skills were measured via mean length of utterance in words (MLUw) and percent correct use of finite verb morphology in obligatory contexts based on spontaneous conversational…

Memory for faces and objects by deaf and hearing signers and hearing nonsigners.

PubMed

Arnold, P; Murray, C

1998-07-01

The memory of 11 deaf and 11 hearing British Sign Language users and 11 hearing nonsigners for pictures of faces of and verbalizable objects was measured using the game Concentration. The three groups performed at the same level for the objects. In contrast the deaf signers were better for faces than the hearing signers, who in turn were superior to the hearing nonsigners, who were the worst. Three hypotheses were made: That there would be no significant difference in terms of the number of attempts between the three groups on the verbalizable object task, that the hearing and deaf signers would demonstrate superior performance to that of the hearing nonsigners on the matching faces task, and that the hearing and deaf signers would exhibit similar performance levels on the matching faces task. The first two hypotheses were supported, but the third was not. Deaf signers were found to be superior for memory for faces to hearing signers and hearing nonsigners. Possible explanations for the findings are discussed, including the possibility that deafness and the long use of sign language have additive effects.

Low empathy in deaf and hard of hearing (pre)adolescents compared to normal hearing controls.

PubMed

Netten, Anouk P; Rieffe, Carolien; Theunissen, Stephanie C P M; Soede, Wim; Dirks, Evelien; Briaire, Jeroen J; Frijns, Johan H M

2015-01-01

The purpose of this study was to examine the level of empathy in deaf and hard of hearing (pre)adolescents compared to normal hearing controls and to define the influence of language and various hearing loss characteristics on the development of empathy. The study group (mean age 11.9 years) consisted of 122 deaf and hard of hearing children (52 children with cochlear implants and 70 children with conventional hearing aids) and 162 normal hearing children. The two groups were compared using self-reports, a parent-report and observation tasks to rate the children's level of empathy, their attendance to others' emotions, emotion recognition, and supportive behavior. Deaf and hard of hearing children reported lower levels of cognitive empathy and prosocial motivation than normal hearing children, regardless of their type of hearing device. The level of emotion recognition was equal in both groups. During observations, deaf and hard of hearing children showed more attention to the emotion evoking events but less supportive behavior compared to their normal hearing peers. Deaf and hard of hearing children attending mainstream education or using oral language show higher levels of cognitive empathy and prosocial motivation than deaf and hard of hearing children who use sign (supported) language or attend special education. However, they are still outperformed by normal hearing children. Deaf and hard of hearing children, especially those in special education, show lower levels of empathy than normal hearing children, which can have consequences for initiating and maintaining relationships.

Validity of hearing impairment calculation methods for prediction of self-reported hearing handicap.

PubMed

John, Andrew B; Kreisman, Brian M; Pallett, Stephen

2012-01-01

Worker's compensation for hearing loss caused by occupational noise exposure is calculated by varying methods, from state to state within the United States (US), with many employing arithmetic formulas based on the pure-tone audiogram, to quantify hearing loss. Several assumptions unsupported or weakly supported by empirical data underlie these formulas. The present study evaluated the ability of various arithmetic hearing impairment calculations to predict a self-reported hearing handicap in a sample of presenting with sensorineural hearing loss. 204 adults (127 male, 77 female) ranging in age from 18 to 94 served as participants. The sample was selected to exclude patients who had been referred for hearing testing for a medicolegal examination or a hearing conservation appointment. A hearing handicap was measured by the Hearing Handicap Inventory for Adults/for the Elderly (HHIA/E). The covariance analysis of linear structural equations was used to assess the relative strength of correlation with the HHIA/E score among the six formulas and various forms of pure-tone average. The results revealed that all the hearing impairment calculations examined were significantly, but weakly, correlated with the self-reported hearing impairment scores. No significant differences among the predictive abilities of the impairment calculations were evident; however, the average binaural impairment assigned differed significantly among the six calculations examined. Individuals who demonstrated 0% impairment had significantly lower (i.e., better) HHIA/E scores compared to those with non-zero impairment for each formula. These results supported the idea that audiometric data provided an insufficient explanation for real-world hearing difficulties.

Rehabilitation of Hearing.

ERIC Educational Resources Information Center

World Federation of the Deaf, Rome (Italy).

Rehabilitation of hearing is considered in five conference papers. Two papers come from Poland: "Rehabilitation of Hearing in Children 'Deaf' in First 5 Years of Age" by D. Borkowska-Gaertig and others and "Possibilities of Hearing Improvement in Adults with Conservative Methods" by T. Bystrzanowska. Also included are…

Implementing Hearing Safety

ERIC Educational Resources Information Center

Cliffe, Roger

1978-01-01

Hearing damage from noise exposure and approaches to implementing hearing safety in school industrial laboratories through noise reduction and protective equipment are discussed. Although all states have not adopted the Occupational Safety and Health Act, teachers should be aware of noise hazards and act to protect hearing. (MF)

Hearing loss and the central auditory system: Implications for hearing aids

NASA Astrophysics Data System (ADS)

Frisina, Robert D.

2003-04-01

Hearing loss can result from disorders or damage to the ear (peripheral auditory system) or the brain (central auditory system). Here, the basic structure and function of the central auditory system will be highlighted as relevant to cases of permanent hearing loss where assistive devices (hearing aids) are called for. The parts of the brain used for hearing are altered in two basic ways in instances of hearing loss: (1) Damage to the ear can reduce the number and nature of input channels that the brainstem receives from the ear, causing plasticity of the central auditory system. This plasticity may partially compensate for the peripheral loss, or add new abnormalities such as distorted speech processing or tinnitus. (2) In some situations, damage to the brain can occur independently of the ear, as may occur in cases of head trauma, tumors or aging. Implications of deficits to the central auditory system for speech perception in noise, hearing aid use and future innovative circuit designs will be provided to set the stage for subsequent presentations in this special educational session. [Work supported by NIA-NIH Grant P01 AG09524 and the International Center for Hearing & Speech Research, Rochester, NY.

Postural control assessment in students with normal hearing and sensorineural hearing loss.

PubMed

Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2015-01-01

Children with sensorineural hearing loss can present with instabilities in postural control, possibly as a consequence of hypoactivity of their vestibular system due to internal ear injury. To assess postural control stability in students with normal hearing (i.e., listeners) and with sensorineural hearing loss, and to compare data between groups, considering gender and age. This cross-sectional study evaluated the postural control of 96 students, 48 listeners and 48 with sensorineural hearing loss, aged between 7 and 18 years, of both genders, through the Balance Error Scoring Systems scale. This tool assesses postural control in two sensory conditions: stable surface and unstable surface. For statistical data analysis between groups, the Wilcoxon test for paired samples was used. Students with hearing loss showed more instability in postural control than those with normal hearing, with significant differences between groups (stable surface, unstable surface) (p<0.001). Students with sensorineural hearing loss showed greater instability in the postural control compared to normal hearing students of the same gender and age. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Low Empathy in Deaf and Hard of Hearing (Pre)Adolescents Compared to Normal Hearing Controls

PubMed Central

Netten, Anouk P.; Rieffe, Carolien; Theunissen, Stephanie C. P. M.; Soede, Wim; Dirks, Evelien; Briaire, Jeroen J.; Frijns, Johan H. M.

2015-01-01

Objective The purpose of this study was to examine the level of empathy in deaf and hard of hearing (pre)adolescents compared to normal hearing controls and to define the influence of language and various hearing loss characteristics on the development of empathy. Methods The study group (mean age 11.9 years) consisted of 122 deaf and hard of hearing children (52 children with cochlear implants and 70 children with conventional hearing aids) and 162 normal hearing children. The two groups were compared using self-reports, a parent-report and observation tasks to rate the children’s level of empathy, their attendance to others’ emotions, emotion recognition, and supportive behavior. Results Deaf and hard of hearing children reported lower levels of cognitive empathy and prosocial motivation than normal hearing children, regardless of their type of hearing device. The level of emotion recognition was equal in both groups. During observations, deaf and hard of hearing children showed more attention to the emotion evoking events but less supportive behavior compared to their normal hearing peers. Deaf and hard of hearing children attending mainstream education or using oral language show higher levels of cognitive empathy and prosocial motivation than deaf and hard of hearing children who use sign (supported) language or attend special education. However, they are still outperformed by normal hearing children. Conclusions Deaf and hard of hearing children, especially those in special education, show lower levels of empathy than normal hearing children, which can have consequences for initiating and maintaining relationships. PMID:25906365

Music and Hearing Aids

PubMed Central

Moore, Brian C. J.

2014-01-01

The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. PMID:25361601

Music and hearing aids.

PubMed

Madsen, Sara M K; Moore, Brian C J

2014-10-31

The signal processing and fitting methods used for hearing aids have mainly been designed to optimize the intelligibility of speech. Little attention has been paid to the effectiveness of hearing aids for listening to music. Perhaps as a consequence, many hearing-aid users complain that they are not satisfied with their hearing aids when listening to music. This issue inspired the Internet-based survey presented here. The survey was designed to identify the nature and prevalence of problems associated with listening to live and reproduced music with hearing aids. Responses from 523 hearing-aid users to 21 multiple-choice questions are presented and analyzed, and the relationships between responses to questions regarding music and questions concerned with information about the respondents, their hearing aids, and their hearing loss are described. Large proportions of the respondents reported that they found their hearing aids to be helpful for listening to both live and reproduced music, although less so for the former. The survey also identified problems such as distortion, acoustic feedback, insufficient or excessive gain, unbalanced frequency response, and reduced tone quality. The results indicate that the enjoyment of listening to music with hearing aids could be improved by an increase of the input and output dynamic range, extension of the low-frequency response, and improvement of feedback cancellation and automatic gain control systems. © The Author(s) 2014.

Auditory Outcomes with Hearing Rehabilitation in Children with Unilateral Hearing Loss: A Systematic Review.

PubMed

Appachi, Swathi; Specht, Jessica L; Raol, Nikhila; Lieu, Judith E C; Cohen, Michael S; Dedhia, Kavita; Anne, Samantha

2017-10-01

Objective Options for management of unilateral hearing loss (UHL) in children include conventional hearing aids, bone-conduction hearing devices, contralateral routing of signal (CROS) aids, and frequency-modulating (FM) systems. The objective of this study was to systematically review the current literature to characterize auditory outcomes of hearing rehabilitation options in UHL. Data Sources PubMed, EMBASE, Medline, CINAHL, and Cochrane Library were searched from inception to January 2016. Manual searches of bibliographies were also performed. Review Methods Studies analyzing auditory outcomes of hearing amplification in children with UHL were included. Outcome measures included functional and objective auditory results. Two independent reviewers evaluated each abstract and article. Results Of the 249 articles identified, 12 met inclusion criteria. Seven articles solely focused on outcomes with bone-conduction hearing devices. Outcomes favored improved pure-tone averages, speech recognition thresholds, and sound localization in implanted patients. Five studies focused on FM systems, conventional hearing aids, or CROS hearing aids. Limited data are available but suggest a trend toward improvement in speech perception with hearing aids. FM systems were shown to have the most benefit for speech recognition in noise. Studies evaluating CROS hearing aids demonstrated variable outcomes. Conclusions Data evaluating functional and objective auditory measures following hearing amplification in children with UHL are limited. Most studies do suggest improvement in speech perception, speech recognition in noise, and sound localization with a hearing rehabilitation device.

Hearing loss

MedlinePlus

... eardrum) Ringing or buzzing sound in the ears ( tinnitus ) Causes Conductive hearing loss (CHL) occurs because of ... severe hearing loss or ringing in the ears (tinnitus). You have other symptoms, such as ear pain , ...

Hearing Aids

MedlinePlus

... primarily useful in improving the hearing and speech comprehension of people who have hearing loss that results ... and you can change the program for different listening environments—from a small, quiet room to a ...

Applications of direct-to-consumer hearing devices for adults with hearing loss: a review

PubMed Central

Manchaiah, Vinaya; Taylor, Brian; Dockens, Ashley L; Tran, Nicole R; Lane, Kayla; Castle, Mariana; Grover, Vibhu

2017-01-01

Background This systematic literature review is aimed at investigating applications of direct-to-consumer hearing devices for adults with hearing loss. This review discusses three categories of direct-to-consumer hearing devices: 1) personal sound amplification products (PSAPs), 2) direct-mail hearing aids, and 3) over-the-counter (OTC) hearing aids. Method A literature review was conducted using EBSCOhost and included the databases CINAHL, MEDLINE, and PsycINFO. After applying prior agreed inclusion and exclusion criteria, 13 reports were included in the review. Results Included studies fell into three domains: 1) electroacoustic characteristics, 2) consumer surveys, and 3) outcome evaluations. Electroacoustic characteristics of these devices vary significantly with some meeting the stringent acoustic criteria used for hearing aids, while others producing dangerous output levels (ie, over 120-dB sound pressure level). Low-end (or low-cost) devices were typically poor in acoustic quality and did not meet gain levels necessary for most adult and elderly hearing loss patterns (eg, presbycusis), especially in high frequencies. Despite direct-mail hearing aids and PSAPs being associated with lower satisfaction when compared to hearing aids purchased through hearing health care professionals, consumer surveys suggest that 5%–19% of people with hearing loss purchase hearing aids through direct-mail or online. Studies on outcome evaluation suggest positive outcomes of OTC devices in the elderly population. Of note, OTC outcomes appear better when a hearing health care professional supports these users. Conclusion While some direct-to-consumer hearing devices have the capability to produce adverse effects due to production of dangerously high sound levels and internal noise, the existing literature suggests that there are potential benefits of these devices. Research of direct-to-consumer hearing devices is limited, and current published studies are of weak quality. Much

20 CFR 220.56 - Securing medical evidence at the hearings officer hearing level.

Code of Federal Regulations, 2010 CFR

2010-04-01

... medical evidence at the hearings officer hearing level. (a) Where there is a conflict in the medical... reasoning will be explained in the decision rationale. Where such resolution is not possible, the hearings..., prognosis, etc.) to resolve the conflict. Even in the absence of a conflict, the hearings officer will also...

37 CFR 11.44 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 37 Patents, Trademarks, and Copyrights 1 2012-07-01 2012-07-01 false Hearings. 11.44 Section 11.44... Proceedings; Jurisdiction, Sanctions, Investigations, and Proceedings § 11.44 Hearings. (a) The hearing officer shall preside over hearings in disciplinary proceedings. The hearing officer shall set the time...

37 CFR 11.44 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 37 Patents, Trademarks, and Copyrights 1 2014-07-01 2014-07-01 false Hearings. 11.44 Section 11.44... Proceedings; Jurisdiction, Sanctions, Investigations, and Proceedings § 11.44 Hearings. (a) The hearing officer shall preside over hearings in disciplinary proceedings. The hearing officer shall set the time...

37 CFR 11.44 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 37 Patents, Trademarks, and Copyrights 1 2010-07-01 2010-07-01 false Hearings. 11.44 Section 11.44... Proceedings; Jurisdiction, Sanctions, Investigations, and Proceedings § 11.44 Hearings. (a) The hearing officer shall preside over hearings in disciplinary proceedings. The hearing officer shall set the time...

Hearing impairment and retirement.

PubMed

Fischer, Mary E; Cruickshanks, Karen J; Pinto, Alex; Klein, Barbara E K; Klein, Ronald; Dalton, Dayna S

2014-02-01

Many factors influence the decision to retire including age, insurance, and pension availability along with physical and mental health. Hearing impairment may be one such factor. The purpose of this study was to compare the 15 yr retirement rate among subjects with and without hearing impairment. Prospective, population-based study. Subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a longitudinal investigation of age-related hearing loss. Participants who were working full- or part-time in 1993-1995 were included (n = 1410, mean age = 57.8 yr). Data from four EHLS phases (1993-1995, 1998-2000, 2003-2005, and 2009-2010) were analyzed in 2010-2012. Hearing impairment was defined as a pure tone threshold average (at 0.5, 1, 2, and 4 kHz) greater than 25 dB HL in the worse ear. Employment status was determined at each of the four phases. Kaplan-Meier estimates of the cumulative incidence of retirement were calculated, and Cox discrete-time modeling was used to determine the effect of hearing impairment on the rate of retirement. The cumulative incidence of retirement was significantly (p < 0.02) higher in those with a hearing impairment (77%) compared to those without a hearing impairment (74%). After adjustment for age, gender, self-reported health, and history of chronic disease, there was no significant difference in the rate of retirement between those with and without a hearing impairment (hazard ratio [HR] = 0.9, 95% confidence interval (CI) = 0.7, 1.1). Similar results were observed when hearing aid users were excluded, when hearing impairment was based on the better ear thresholds, and when analyses were restricted to those under 65 yr of age and working full-time at baseline. Participants with a hearing impairment were less likely to state that the main reason for retirement was that the time seemed right. Hearing impairment was found to be associated with a higher rate of retirement, but the association was not independent of the

The Prevalence of Gap Junction Protein Beta 2 (GJB2) Mutations in Non Syndromic Sensorineural Hearing Loss in Çukurova Region.

PubMed

Bozdoğan, Sevcan Tuğ; Kuran, Gökhan; Yüregir, Özge Özalp; Aslan, Hüseyin; Haytoğlu, Süheyl; Ayaz, Akif; Arıkan, Osman Kürşat

2015-08-01

To date, studies in all populations showed that mutations in the gene of Gap junction protein beta 2 (GJB2) play an important role in non-syndromic autosomal recessive congenital hearing loss. The aim of this study was to evaluate GJB2 gene of patients with hearing loss in our region using deoxyribonucleic acid (DNA) sequencing method and to demonstrate region-specific mutation and polymorphism distribution. Patients who had bilateral severe sensorineural non-syndromic hearing loss identified by audiologic evaluation were included. Peripheral blood samples were collected and the GJB2 gene exon1 and exon 2 regions were amplified by polymerase chain reaction (PCR). Obtained PCR products were sequenced by the DNA sequence analysis method (SeqFinder Sequencing System; ABI 3130; Foster City, CA, USA) and analyzed using the SeqScape software. Of the 77 patients, 16 had homozygous or heterozygous mutation. The mutation of 35delG, which is known as the most frequent mutation of GJB2 gene, was also the most frequently seen mutation at a ratio of 5.5% in patients with hearing loss in our region; this was followed by the V27I mutation. As this is the first study conducted by sequence analysis in our region, it was worth to be presented in terms of showing the distribution of mutation.

Hearing and hearing loss: Causes, effects, and treatments

NASA Astrophysics Data System (ADS)

Schmiedt, Richard A.

2003-04-01

Hearing loss can have multiple causes. The outer and middle ears are conductive pathways for acoustic energy to the inner ear (cochlea) and help shape our spectral sensitivity. Conductive hearing loss is mechanical in nature such that the energy transfer to the cochlea is impeded, often from eardrum perforations or middle ear fluid buildup. Beyond the middle ear, the cochlea comprises three interdependent systems necessary for normal hearing. The first is that of basilar-membrane micromechanics including the outer hair cells. This system forms the basis of the cochlear amplifier and is the most vulnerable to noise and drug exposure. The second system comprises the ion pumps in the lateral wall tissues of the cochlea. These highly metabolic cells provide energy to the cochlear amplifier in the form of electrochemical potentials. This second system is particularly vulnerable to the effects of aging. The third system comprises the inner hair cells and their associated sensory nerve fibers. This system is the transduction stage, changing mechanical vibrations to nerve impulses. New treatments for hearing loss are on the horizon; however, at present the best strategy is avoidance of cochlear trauma and the proper use of hearing aids. [Work supported by NIA and MUSC.

Hearing Aid Tester

NASA Technical Reports Server (NTRS)

1978-01-01

Hearing aids often develop malfunctions that are not detected by the wearer. This is particularly true when the wearers are school-age children. Studies of selected groups showed that from 30 to more than 50 percent of school children were not getting adequate benefit from their hearing aids because of unrecognized malfunctions, usually low or dead batteries. This can be serious because hearing impairment retards a child's educational progress. NASA technology incorporated in the Hearing Aid Malfunction Detection Unit (HAMDU), the device pictured, is expected to provide an effective countermeasure to the childrens' hearing aid problem. A patent license has been awarded to a minority-owned firm, Hopkins International Company, a subsidiary of H. H. Aerospace Design Co., Inc., Elmford, New York. The company plans early commercial availability of its version of the device.

Prediction of hearing outcomes by multiple regression analysis in patients with idiopathic sudden sensorineural hearing loss.

PubMed

Suzuki, Hideaki; Tabata, Takahisa; Koizumi, Hiroki; Hohchi, Nobusuke; Takeuchi, Shoko; Kitamura, Takuro; Fujino, Yoshihisa; Ohbuchi, Toyoaki

2014-12-01

This study aimed to create a multiple regression model for predicting hearing outcomes of idiopathic sudden sensorineural hearing loss (ISSNHL). The participants were 205 consecutive patients (205 ears) with ISSNHL (hearing level ≥ 40 dB, interval between onset and treatment ≤ 30 days). They received systemic steroid administration combined with intratympanic steroid injection. Data were examined by simple and multiple regression analyses. Three hearing indices (percentage hearing improvement, hearing gain, and posttreatment hearing level [HLpost]) and 7 prognostic factors (age, days from onset to treatment, initial hearing level, initial hearing level at low frequencies, initial hearing level at high frequencies, presence of vertigo, and contralateral hearing level) were included in the multiple regression analysis as dependent and explanatory variables, respectively. In the simple regression analysis, the percentage hearing improvement, hearing gain, and HLpost showed significant correlation with 2, 5, and 6 of the 7 prognostic factors, respectively. The multiple correlation coefficients were 0.396, 0.503, and 0.714 for the percentage hearing improvement, hearing gain, and HLpost, respectively. Predicted values of HLpost calculated by the multiple regression equation were reliable with 70% probability with a 40-dB-width prediction interval. Prediction of HLpost by the multiple regression model may be useful to estimate the hearing prognosis of ISSNHL. © The Author(s) 2014.

17 CFR 204.64 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 17 Commodity and Securities Exchanges 2 2013-04-01 2013-04-01 false Hearing. 204.64 Section 204.64... Administrative Wage Garnishment § 204.64 Hearing. (a) Request for hearing. The Commission will order a hearing... hearing concerning, for debts not previously established by judicial or administrative order, the...

42 CFR 430.70 - Notice of hearing or opportunity for hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 4 2010-10-01 2010-10-01 false Notice of hearing or opportunity for hearing. 430.70 Section 430.70 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND... Hearings on Conformity of State Medicaid Plans and Practice to Federal Requirements § 430.70 Notice of...

45 CFR 99.11 - Notice of hearing or opportunity for hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 45 Public Welfare 1 2010-10-01 2010-10-01 false Notice of hearing or opportunity for hearing. 99.11 Section 99.11 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION PROCEDURE FOR HEARINGS FOR THE CHILD CARE AND DEVELOPMENT FUND Preliminary Matters-Notice and Parties § 99...

Epigenome-Wide DNA Methylation in Hearing Ability: New Mechanisms for an Old Problem

PubMed Central

Wolber, Lisa E.; Steves, Claire J.; Tsai, Pei-Chien; Deloukas, Panos; Spector, Tim D.

2014-01-01

Epigenetic regulation of gene expression has been shown to change over time and may be associated with environmental exposures in common complex traits. Age-related hearing impairment is a complex disorder, known to be heritable, with heritability estimates of 57–70%. Epigenetic regulation might explain the observed difference in age of onset and magnitude of hearing impairment with age. Epigenetic epidemiology studies using unrelated samples can be limited in their ability to detect small effects, and recent epigenetic findings in twins underscore the power of this well matched study design. We investigated the association between venous blood DNA methylation epigenome-wide and hearing ability. Pure-tone audiometry (PTA) and Illumina HumanMethylation array data were obtained from female twin volunteers enrolled in the TwinsUK register. Two study groups were explored: first, an epigenome-wide association scan (EWAS) was performed in a discovery sample (n = 115 subjects, age range: 47–83 years, Illumina 27 k array), then replication of the top ten associated probes from the discovery EWAS was attempted in a second unrelated sample (n = 203, age range: 41–86 years, Illumina 450 k array). Finally, a set of monozygotic (MZ) twin pairs (n = 21 pairs) within the discovery sample (Illumina 27 k array) was investigated in more detail in an MZ discordance analysis. Hearing ability was strongly associated with DNA methylation levels in the promoter regions of several genes, including TCF25 (cg01161216, p = 6.6×10−6), FGFR1 (cg15791248, p = 5.7×10−5) and POLE (cg18877514, p = 6.3×10−5). Replication of these results in a second sample confirmed the presence of differential methylation at TCF25 (p(replication) = 6×10−5) and POLE (p(replication) = 0.016). In the MZ discordance analysis, twins' intrapair difference in hearing ability correlated with DNA methylation differences at ACP6 (cg01377755, r = −0.75, p = 1.2×10�

36 CFR 1211.620 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 36 Parks, Forests, and Public Property 3 2011-07-01 2011-07-01 false Hearings. 1211.620 Section... Procedures § 1211.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the designated agency official that the matter be scheduled for hearing; or (2) Advise...

28 CFR 42.109 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 28 Judicial Administration 1 2014-07-01 2014-07-01 false Hearings. 42.109 Section 42.109 Judicial....109 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 42... that the responsible Department official schedule the matter for hearing, or (2) advise the applicant...

28 CFR 42.109 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 28 Judicial Administration 1 2013-07-01 2013-07-01 false Hearings. 42.109 Section 42.109 Judicial....109 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 42... that the responsible Department official schedule the matter for hearing, or (2) advise the applicant...

17 CFR 201.300 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 17 Commodity and Securities Exchanges 2 2013-04-01 2013-04-01 false Hearings. 201.300 Section 201... Practice Rules Regarding Hearings § 201.300 Hearings. Hearings for the purpose of taking evidence shall be held only upon order of the Commission. All hearings shall be conducted in a fair, impartial...

49 CFR 209.115 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 4 2011-10-01 2011-10-01 false Hearing. 209.115 Section 209.115 Transportation... Hearing. (a) When a hearing is requested and scheduled under § 209.113, a hearing officer designated by the Chief Counsel convenes and presides over the hearing. If requested by respondent and if...

36 CFR 1211.620 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 36 Parks, Forests, and Public Property 3 2014-07-01 2014-07-01 false Hearings. 1211.620 Section... Procedures § 1211.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the designated agency official that the matter be scheduled for hearing; or (2) Advise...

28 CFR 42.109 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 28 Judicial Administration 1 2011-07-01 2011-07-01 false Hearings. 42.109 Section 42.109 Judicial....109 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 42... that the responsible Department official schedule the matter for hearing, or (2) advise the applicant...

49 CFR 209.115 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 49 Transportation 4 2010-10-01 2010-10-01 false Hearing. 209.115 Section 209.115 Transportation... Hearing. (a) When a hearing is requested and scheduled under § 209.113, a hearing officer designated by the Chief Counsel convenes and presides over the hearing. If requested by respondent and if...

36 CFR 1211.620 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 36 Parks, Forests, and Public Property 3 2010-07-01 2010-07-01 false Hearings. 1211.620 Section... Procedures § 1211.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the designated agency official that the matter be scheduled for hearing; or (2) Advise...

Motivation to Address Self-Reported Hearing Problems in Adults with Normal Hearing Thresholds

ERIC Educational Resources Information Center

Alicea, Carly C. M.; Doherty, Karen A.

2017-01-01

Purpose: The purpose of this study was to compare the motivation to change in relation to hearing problems in adults with normal hearing thresholds but who report hearing problems and that of adults with a mild-to-moderate sensorineural hearing loss. Factors related to their motivation were also assessed. Method: The motivation to change in…

"I know you can hear me": neural correlates of feigned hearing loss.

PubMed

McPherson, Bradley; McMahon, Katie; Wilson, Wayne; Copland, David

2012-08-01

In the assessment of human hearing, it is often important to determine whether hearing loss is organic or nonorganic in nature. Nonorganic, or functional, hearing loss is often associated with deceptive intention on the part of the listener. Over the past decade, functional neuroimaging has been used to study the neural correlates of deception, and studies have consistently highlighted the contribution of the prefrontal cortex in such behaviors. Can patterns of brain activity be similarly used to detect when an individual is feigning a hearing loss? To answer this question, 15 adult participants were requested to respond to pure tones and simple words correctly, incorrectly, randomly, or with the intent to feign a hearing loss. As predicted, more activity was observed in the prefrontal cortices (as measured by functional magnetic resonance imaging), and delayed behavioral reaction times were noted, when the participants feigned a hearing loss or responded randomly versus when they responded correctly or incorrectly. The results suggest that cortical imaging techniques could play a role in identifying individuals who are feigning hearing loss. Copyright © 2011 Wiley Periodicals, Inc.

[Subsidized project of hearing aid purchase for mild-moderate hearing impaired children in Akita prefecture].

PubMed

Sato, Teruyuki; Nakazawa, Misao; Takahashi, Shin; Ishikawa, Kazuo

2013-06-01

The dissemination of newborn hearing screening has detected children with mild-moderate hearing impairment at an early age. However, there is no nation-wide welfare system for children with mild-moderate hearing impairment in Japan. Under these kinds of social conditions, a subsidized project of hearing aid purchase for mild-moderate hearing impaired children has come into force from April 2010 in Akita prefecture. All 18 candidates who applied for this project were subsidized in Akita prefecture. Eighteen children purchased their hearing aids using this subsidy. The feature of this project was that every child could have access to subsidies as long as their doctor recognized the effectiveness of hearing aids because children with hearing impairment need to learn language. They contacted the hospital, prefectural government and institutes related to hearing loss before this project come into force. We recognized parents who are raising a child with mild-moderate hearing impairment have high interest in this project. Hearing aids can represent a considerable expense for young parents who are raising a child. We encountered some children who had to give up the idea of hearing aids due to their parents' economic circumstances before this project become effective. These situations were completely avoided after this project came into being. This administrative purpose was of demonstrated value in children with mild-moderate hearing impairment.

Streptococcus suis meningitis with bilateral sensorineural hearing loss.

PubMed

Huh, Hee Jae; Park, Kyoung-Jin; Jang, Ja-Hyun; Lee, Mina; Lee, Jang Ho; Ahn, Yoon Hee; Kang, Cheol-In; Ki, Chang-Seok; Lee, Nam Yong

2011-07-01

Streptococcus suis infection is an emerging zoonosis in Asia. The most common disease manifestation is meningitis, which is often associated with hearing loss and cochleovestibular signs. S. suis infection in humans mainly occurs among risk groups that have frequent exposure to pigs or raw pork. Here, we report a case of S. suis meningitis in a 67-yr-old pig carcass handler, who presented with dizziness and sensorineural hearing loss followed by headaches. Gram-positive diplococci were isolated from cerebrospinal fluid (CSF) and blood cultures and showed gray-white colonies with α-hemolysis. S. suis was identified from CSF and blood cultures by using a Vitek 2 system (bioMérieux, France), API 20 STREP (bioMérieux), and performing 16S rRNA and tuf gene sequencing. Even after receiving antibiotic treatment, patients with S. suis infection frequently show complications such as hearing impairment and vestibular dysfunction. To the best of our knowledge, this is the first case of S. suis meningitis in Korea. Prevention through public health surveillance is recommended, especially for individuals who have occupational exposures to swine and raw pork.

44 CFR 7.13 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 44 Emergency Management and Assistance 1 2012-10-01 2011-10-01 true Hearings. 7.13 Section 7.13... Programs-General § 7.13 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the responsible agency official that the matter be scheduled for hearing or (2) advise...

22 CFR 512.21 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 22 Foreign Relations 2 2012-04-01 2009-04-01 true Hearing. 512.21 Section 512.21 Foreign Relations....21 Hearing. (a) Petition for hearing. (1) A hearing may be requested by filing a written petition... section, the employee's right to hearing will be considered waived, and salary offset will be implemented...

10 CFR 205.172 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 10 Energy 3 2013-01-01 2013-01-01 false Hearings. 205.172 Section 205.172 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.172 Hearings. (a) The DOE in its discretion may direct that a hearing be convened on its own initiative or upon...

34 CFR 668.116 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 34 Education 3 2011-07-01 2011-07-01 false Hearing. 668.116 Section 668.116 Education Regulations... Program Review Determinations § 668.116 Hearing. (a) A hearing is a process conducted by the hearing official whereby an orderly presentation of arguments and evidence is made by the parties. (b) The hearing...

10 CFR 1003.62 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 10 Energy 4 2011-01-01 2011-01-01 false Hearings. 1003.62 Section 1003.62 Energy DEPARTMENT OF ENERGY (GENERAL PROVISIONS) OFFICE OF HEARINGS AND APPEALS PROCEDURAL REGULATIONS Conferences and Hearings § 1003.62 Hearings. (a) The OHA in its discretion may direct that a hearing be convened on its own...

10 CFR 1003.62 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 10 Energy 4 2012-01-01 2012-01-01 false Hearings. 1003.62 Section 1003.62 Energy DEPARTMENT OF ENERGY (GENERAL PROVISIONS) OFFICE OF HEARINGS AND APPEALS PROCEDURAL REGULATIONS Conferences and Hearings § 1003.62 Hearings. (a) The OHA in its discretion may direct that a hearing be convened on its own...

22 CFR 512.21 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 22 Foreign Relations 2 2013-04-01 2009-04-01 true Hearing. 512.21 Section 512.21 Foreign Relations....21 Hearing. (a) Petition for hearing. (1) A hearing may be requested by filing a written petition... section, the employee's right to hearing will be considered waived, and salary offset will be implemented...

40 CFR 57.807 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 40 Protection of Environment 6 2014-07-01 2014-07-01 false Hearing. 57.807 Section 57.807... § 57.807 Hearing. (a) Composition of hearing panel. The Presiding Officer shall preside at the hearing held under this subpart. An EPA panel shall also take part in the hearing. In general, the membership...

44 CFR 7.13 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 44 Emergency Management and Assistance 1 2013-10-01 2013-10-01 false Hearings. 7.13 Section 7.13... Programs-General § 7.13 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the responsible agency official that the matter be scheduled for hearing or (2) advise...

10 CFR 205.172 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 10 Energy 3 2011-01-01 2011-01-01 false Hearings. 205.172 Section 205.172 Energy DEPARTMENT OF ENERGY OIL ADMINISTRATIVE PROCEDURES AND SANCTIONS Conferences, Hearings, and Public Hearings § 205.172 Hearings. (a) The DOE in its discretion may direct that a hearing be convened on its own initiative or upon...

40 CFR 57.807 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 40 Protection of Environment 6 2012-07-01 2012-07-01 false Hearing. 57.807 Section 57.807... § 57.807 Hearing. (a) Composition of hearing panel. The Presiding Officer shall preside at the hearing held under this subpart. An EPA panel shall also take part in the hearing. In general, the membership...

10 CFR 2.1405 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 10 Energy 1 2014-01-01 2014-01-01 false Hearing. 2.1405 Section 2.1405 Energy NUCLEAR REGULATORY COMMISSION AGENCY RULES OF PRACTICE AND PROCEDURE Expedited Proceedings with Oral Hearings § 2.1405 Hearing... officer shall hold a hearing on any contention that remains in dispute. At the beginning of the hearing...

44 CFR 7.13 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 44 Emergency Management and Assistance 1 2014-10-01 2014-10-01 false Hearings. 7.13 Section 7.13... Programs-General § 7.13 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the responsible agency official that the matter be scheduled for hearing or (2) advise...

10 CFR 1003.62 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 10 Energy 4 2013-01-01 2013-01-01 false Hearings. 1003.62 Section 1003.62 Energy DEPARTMENT OF ENERGY (GENERAL PROVISIONS) OFFICE OF HEARINGS AND APPEALS PROCEDURAL REGULATIONS Conferences and Hearings § 1003.62 Hearings. (a) The OHA in its discretion may direct that a hearing be convened on its own...

34 CFR 668.88 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 34 Education 3 2011-07-01 2011-07-01 false Hearing. 668.88 Section 668.88 Education Regulations of... Proceedings § 668.88 Hearing. (a) A hearing is an orderly presentation of arguments and evidence conducted by a hearing official. (b) If the hearing official, the designated department official who brought a...

22 CFR 512.21 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 22 Foreign Relations 2 2014-04-01 2014-04-01 false Hearing. 512.21 Section 512.21 Foreign... Offset § 512.21 Hearing. (a) Petition for hearing. (1) A hearing may be requested by filing a written... section, the employee's right to hearing will be considered waived, and salary offset will be implemented...

34 CFR 668.88 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 34 Education 3 2010-07-01 2010-07-01 false Hearing. 668.88 Section 668.88 Education Regulations of... Proceedings § 668.88 Hearing. (a) A hearing is an orderly presentation of arguments and evidence conducted by a hearing official. (b) If the hearing official, the designated department official who brought a...

19 CFR 356.23 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearing. 356.23 Section 356.23 Customs Duties... § 356.23 Hearing. (a) Scheduling of hearing. The administrative law judge will schedule the hearing at a... parties adequately to prepare for the hearing and the importance of expeditiously resolving the matter. (b...

44 CFR 7.13 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 44 Emergency Management and Assistance 1 2010-10-01 2010-10-01 false Hearings. 7.13 Section 7.13... Programs-General § 7.13 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the responsible agency official that the matter be scheduled for hearing or (2) advise...

34 CFR 668.116 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 34 Education 3 2010-07-01 2010-07-01 false Hearing. 668.116 Section 668.116 Education Regulations... Program Review Determinations § 668.116 Hearing. (a) A hearing is a process conducted by the hearing official whereby an orderly presentation of arguments and evidence is made by the parties. (b) The hearing...

The right not to hear: the ethics of parental refusal of hearing rehabilitation.

PubMed

Byrd, Serena; Shuman, Andrew G; Kileny, Sharon; Kileny, Paul R

2011-08-01

To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. Case study with medical ethical discussion and review. Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with Child Protective Services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. The ethics of parental rights to refuse hearing rehabilitation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical, and legal issues is crucial prior to intervening in such challenging situations. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

The Right Not To Hear: The Ethics of Parental Refusal of Hearing Rehabilitation

PubMed Central

Byrd, Serena; Shuman, Andrew G.; Kileny, Sharon; Kileny, Paul R.

2015-01-01

Objective To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. Study Design Case study with medical ethical discussion and review. Methods Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. Discussion This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with child protective services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. Conclusions The ethics of parental rights to refuse hearing augmentation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical and legal issues is crucial prior to intervening in such challenging situations. PMID:21792972

Audibility of reverse alarms under hearing protectors for normal and hearing-impaired listeners.

PubMed

Robinson, G S; Casali, J G

1995-11-01

The question of whether or not an individual suffering from a hearing loss is capable of hearing an auditory alarm or warning is an extremely important industrial safety issue. The ISO Standard that addresses auditory warnings for workplaces requires that any auditory alarm or warning be audible to all individuals in the workplace including those suffering from a hearing loss and/or wearing hearing protection devices (HPDs). Research was undertaken to determine how the ability to detect an alarm or warning signal changed for individuals with normal hearing and two levels of hearing loss as the levels of masking noise and alarm were manipulated. Pink noise was used as the masker and a heavy-equipment reverse alarm was used as the signal. The rating method paradigm of signal detection theory was used as the experimental procedure to separate the subjects' absolute sensitivities to the alarm from their individual criteria for deciding to respond in an affirmative manner. Results indicated that even at a fairly low signal-to-noise ratio (0 dB), subjects with a substantial hearing loss [a pure-tone average (PTA) hearing level of 45-50 dBHL in both ears] were capable of hearing the reverse alarm while wearing a high-attenuation earmuff in the pink noise used in the study.

Pre-enlistment hearing loss and hearing loss disability among US soldiers and marines.

PubMed

Gubata, Marlene E; Packnett, Elizabeth R; Feng, Xiaoshu; Cowan, David N; Niebuhr, David W

2013-01-01

Hearing loss is a common condition among US adults, with some evidence of increasing prevalence in young adults. Noise-induced hearing loss attributable to employment is a significant source of preventable morbidity world-wide. The US military population is largely comprised of young adult males serving in a wide variety of occupations, many in high noise-level conditions, at least episodically. To identify accession and service-related risk factors for hearing-related disability, matched case-control study of US military personnel was conducted. Individuals evaluated for hearing loss disability in the US Army and Marine Corps were frequency matched to controls without history of disability evaluation on service and enlistment year. Conditional logistic regression was used to examine the association between accession and service-related factors and hearing-related disability evaluations between October 2002 and September 2010. Individuals with medically disqualifying audiograms or hearing loss diagnoses at application for military service were 8 and 4 times more likely, respectively, to have a disability evaluation related to hearing loss, after controlling for relevant accession, demographic, and service-related factors. Conservative hearing loss thresholds on pre-enlistment audiograms, stricter hearing loss medical waiver policies or qualified baseline audiograms pre-enlistment are needed in the U.S military. Industrial corporations or labor unions may also benefit from identifying individuals with moderate hearing loss at the time of employment to ensure use of personal protective equipment and engineer controls of noise.

29 CFR 1450.22 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 4 2012-07-01 2012-07-01 false Hearing. 1450.22 Section 1450.22 Labor Regulations Relating... STATES Salary Offset § 1450.22 Hearing. (a) Petition for hearing. (1) A hearing may be requested by..., and is not accepted pursuant to paragraph (a)(4) of this section, the employee's right to hearing will...

29 CFR 2700.108 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 9 2012-07-01 2012-07-01 false Hearing. 2700.108 Section 2700.108 Labor Regulations... Proceedings § 2700.108 Hearing. (a) Procedures. As soon as practicable after the conclusion of the pre-hearing conference, the Judge will hold a hearing on any issue that remains in dispute. The hearing will be in...

45 CFR 1203.9 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 45 Public Welfare 4 2011-10-01 2011-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question has...

45 CFR 1203.9 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 45 Public Welfare 4 2014-10-01 2014-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question has...

29 CFR 1450.22 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 4 2014-07-01 2014-07-01 false Hearing. 1450.22 Section 1450.22 Labor Regulations Relating... STATES Salary Offset § 1450.22 Hearing. (a) Petition for hearing. (1) A hearing may be requested by..., and is not accepted pursuant to paragraph (a)(4) of this section, the employee's right to hearing will...

10 CFR 431.426 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 10 Energy 3 2011-01-01 2011-01-01 false Hearing. 431.426 Section 431.426 Energy DEPARTMENT OF... § 431.426 Hearing. The Secretary may hold a public hearing, and publish notice in the Federal Register of the date and location of the hearing, when he determines that such a hearing is necessary and...

7 CFR 400.132 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 7 Agriculture 6 2012-01-01 2012-01-01 false Hearings. 400.132 Section 400.132 Agriculture... Years § 400.132 Hearings. (a) If an employee timely files a petition for a hearing, the FCIC Official will select the date, time, and location for the hearing. (b) The hearing shall be conducted by an...

18 CFR 1302.9 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 18 Conservation of Power and Water Resources 2 2014-04-01 2014-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or (2...

5 CFR 1215.5 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 5 Administrative Personnel 3 2013-01-01 2013-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to offset. (2) A hearing may be requested by...

45 CFR 1203.9 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 45 Public Welfare 4 2013-10-01 2013-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question has...

5 CFR 1215.5 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 5 Administrative Personnel 3 2014-01-01 2014-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to offset. (2) A hearing may be requested by...

5 CFR 1215.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 5 Administrative Personnel 3 2012-01-01 2012-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to offset. (2) A hearing may be requested by...

7 CFR 400.132 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 7 Agriculture 6 2011-01-01 2011-01-01 false Hearings. 400.132 Section 400.132 Agriculture... Years § 400.132 Hearings. (a) If an employee timely files a petition for a hearing, the FCIC Official will select the date, time, and location for the hearing. (b) The hearing shall be conducted by an...

10 CFR 431.426 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 10 Energy 3 2013-01-01 2013-01-01 false Hearing. 431.426 Section 431.426 Energy DEPARTMENT OF... § 431.426 Hearing. The Secretary may hold a public hearing, and publish notice in the Federal Register of the date and location of the hearing, when he determines that such a hearing is necessary and...

10 CFR 16.9 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 10 Energy 1 2014-01-01 2014-01-01 false Hearing. 16.9 Section 16.9 Energy NUCLEAR REGULATORY... § 16.9 Hearing. (a) Request for hearing. (1) An employee shall file a petition for a hearing in... creditor agency, a hearing may be requested by filing a written petition stating why the employee disputes...

32 CFR 195.10 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 32 National Defense 2 2013-07-01 2013-07-01 false Hearings. 195.10 Section 195.10 National Defense... RIGHTS ACT OF 1964 § 195.10 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing... recipient may request of the responsible Department official that the matter be scheduled for hearing or (2...

18 CFR 1302.9 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 18 Conservation of Power and Water Resources 2 2012-04-01 2012-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or (2...

32 CFR 195.10 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 32 National Defense 2 2011-07-01 2011-07-01 false Hearings. 195.10 Section 195.10 National Defense... RIGHTS ACT OF 1964 § 195.10 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing... recipient may request of the responsible Department official that the matter be scheduled for hearing or (2...

29 CFR 2700.108 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 9 2013-07-01 2013-07-01 false Hearing. 2700.108 Section 2700.108 Labor Regulations... Proceedings § 2700.108 Hearing. (a) Procedures. As soon as practicable after the conclusion of the pre-hearing conference, the Judge will hold a hearing on any issue that remains in dispute. The hearing will be in...

18 CFR 1302.9 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 18 Conservation of Power and Water Resources 2 2013-04-01 2012-04-01 true Hearings. 1302.9 Section... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or (2...

5 CFR 1215.5 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 5 Administrative Personnel 3 2011-01-01 2011-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to offset. (2) A hearing may be requested by...

18 CFR 1302.9 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 18 Conservation of Power and Water Resources 2 2011-04-01 2011-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or (2...

5 CFR 1215.5 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 5 Administrative Personnel 3 2010-01-01 2010-01-01 false Hearing. 1215.5 Section 1215.5... § 1215.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to offset. (2) A hearing may be requested by...

38 CFR 39.12 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 2 2010-07-01 2010-07-01 false Hearings. 39.12 Section... Requirements and Procedures § 39.12 Hearings. (a) No application for a grant to establish, expand, or improve a... hearing. (b) Whenever a hearing is requested under this section, notice of the hearing, procedure for the...

45 CFR 1203.9 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 45 Public Welfare 4 2010-10-01 2010-10-01 false Hearings. 1203.9 Section 1203.9 Public Welfare... Hearings. (a) Opportunity for hearing. When an opportunity for a hearing is required by § 1203.8(c... matter be scheduled for hearing; or (2) Advise the applicant or recipient that the matter in question has...

6 CFR 21.15 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 6 Domestic Security 1 2010-01-01 2010-01-01 false Hearings. 21.15 Section 21.15 Domestic Security... HOMELAND SECURITY § 21.15 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is... may request of the Secretary that the matter be scheduled for hearing; or (2) Advise the applicant or...

10 CFR 16.9 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 10 Energy 1 2010-01-01 2010-01-01 false Hearing. 16.9 Section 16.9 Energy NUCLEAR REGULATORY... § 16.9 Hearing. (a) Request for hearing. (1) An employee shall file a petition for a hearing in... creditor agency, a hearing may be requested by filing a written petition stating why the employee disputes...

18 CFR 1302.9 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 18 Conservation of Power and Water Resources 2 2010-04-01 2010-04-01 false Hearings. 1302.9... Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1302.7(b... notice within which the recipient may request of TVA that the matter be scheduled for hearing or (2...

45 CFR 213.11 - Notice of hearing or opportunity for hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 45 Public Welfare 2 2010-10-01 2010-10-01 false Notice of hearing or opportunity for hearing. 213.11 Section 213.11 Public Welfare Regulations Relating to Public Welfare OFFICE OF FAMILY ASSISTANCE (ASSISTANCE PROGRAMS), ADMINISTRATION FOR CHILDREN AND FAMILIES, DEPARTMENT OF HEALTH AND HUMAN SERVICES PRACTICE AND PROCEDURE FOR HEARINGS TO STATE...

Hearing in Insects.

PubMed

Göpfert, Martin C; Hennig, R Matthias

2016-01-01

Insect hearing has independently evolved multiple times in the context of intraspecific communication and predator detection by transforming proprioceptive organs into ears. Research over the past decade, ranging from the biophysics of sound reception to molecular aspects of auditory transduction to the neuronal mechanisms of auditory signal processing, has greatly advanced our understanding of how insects hear. Apart from evolutionary innovations that seem unique to insect hearing, parallels between insect and vertebrate auditory systems have been uncovered, and the auditory sensory cells of insects and vertebrates turned out to be evolutionarily related. This review summarizes our current understanding of insect hearing. It also discusses recent advances in insect auditory research, which have put forward insect auditory systems for studying biological aspects that extend beyond hearing, such as cilium function, neuronal signal computation, and sensory system evolution.

Predictors of Hearing-Aid Outcomes

PubMed Central

Johannesen, Peter T.; Pérez-González, Patricia; Blanco, José L.; Kalluri, Sridhar; Edwards, Brent

2017-01-01

Over 360 million people worldwide suffer from disabling hearing loss. Most of them can be treated with hearing aids. Unfortunately, performance with hearing aids and the benefit obtained from using them vary widely across users. Here, we investigate the reasons for such variability. Sixty-eight hearing-aid users or candidates were fitted bilaterally with nonlinear hearing aids using standard procedures. Treatment outcome was assessed by measuring aided speech intelligibility in a time-reversed two-talker background and self-reported improvement in hearing ability. Statistical predictive models of these outcomes were obtained using linear combinations of 19 predictors, including demographic and audiological data, indicators of cochlear mechanical dysfunction and auditory temporal processing skills, hearing-aid settings, working memory capacity, and pretreatment self-perceived hearing ability. Aided intelligibility tended to be better for younger hearing-aid users with good unaided intelligibility in quiet and with good temporal processing abilities. Intelligibility tended to improve by increasing amplification for low-intensity sounds and by using more linear amplification for high-intensity sounds. Self-reported improvement in hearing ability was hard to predict but tended to be smaller for users with better working memory capacity. Indicators of cochlear mechanical dysfunction, alone or in combination with hearing settings, did not affect outcome predictions. The results may be useful for improving hearing aids and setting patients’ expectations. PMID:28929903

Public awareness of audiology, hearing and hearing health in the Limpopo Province, South Africa

PubMed Central

Sebothoma, Ben; Kgare, Khomotjo S.

2017-01-01

Background The burden of hearing loss is on the increase, especially in low-income countries such as South Africa. The need for urgent action to prevent ear and hearing problems is a priority, especially as in many cases permanent hearing loss is preventable. In South Africa, as in other developing countries, there is a limited number of hearing health professionals and audiological resources. The lack of hearing health services may impact the general public’s awareness of hearing and hearing health. Limited information is available on the South African public’s knowledge of audiologists and the services they provide, especially in underserved rural communities. Aim The aim of this study was to describe individuals’ awareness of the audiology profession, hearing and hearing loss, and hearing health in a rural area of the Limpopo Province. Method A cross-sectional survey design was employed for the purpose of this study. Using a random sampling strategy, 297 households in four rural villages were selected and a self-developed questionnaire was administered to one individual (18 years and older) per household. The questionnaire consisted of 23 questions targeting awareness of the audiology profession, as well as knowledge of hearing, hearing loss and hearing health. Results Only 14% of participants were aware of the audiology profession, indicating that individuals living in rural communities are not aware of the role of audiologists and the services they provide. Doctors and nurses were identified by participants as the individuals who assist them with hearing-related problems. Although most participants (87%) acknowledged that it is very important to undergo a hearing test, only 5% have previously visited an audiologist. Most participants were aware that ear infections and excessive noise exposure can cause hearing loss. The majority also believed that ears must be kept clean at all times and used cotton buds to maintain ear hygiene. Conclusion There is a

12 CFR 308.155 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearing. 308.155 Section 308.155 Banks and... Pursuant to Section 32 of the FDIA § 308.155 Hearing. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for a hearing filed pursuant...

12 CFR 308.164 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 12 Banks and Banking 5 2012-01-01 2012-01-01 false Hearings. 308.164 Section 308.164 Banks and... Where a Felony ls Charged § 308.164 Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for hearing filed pursuant to § 308.163...

12 CFR 308.164 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearings. 308.164 Section 308.164 Banks and... Where a Felony ls Charged § 308.164 Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for hearing filed pursuant to § 308.163...

39 CFR 957.13 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 39 Postal Service 1 2013-07-01 2013-07-01 false Hearings. 957.13 Section 957.13 Postal Service... SUSPENSION FROM CONTRACTING § 957.13 Hearings. (a) Hearings are held at 2101 Wilson Boulevard, Suite 600... than 7 days prior to the scheduled date of a hearing, file a request that such hearing be held at a...

45 CFR 16.11 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 45 Public Welfare 1 2012-10-01 2012-10-01 false Hearing. 16.11 Section 16.11 Public Welfare... BOARD § 16.11 Hearing. (a) Electing a hearing. If the appellant believes a hearing is appropriate, the... appeal file). The Board will approve a request (and may schedule a hearing on its own or in response to a...

14 CFR 13.79 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 14 Aeronautics and Space 1 2014-01-01 2014-01-01 false Hearing. 13.79 Section 13.79 Aeronautics....79 Hearing. If an alleged violator requests a hearing in accordance with § 13.75, the procedure of subpart D of this part applies. At the close of the hearing, the Hearing Officer, on the record or...

15 CFR 8.12 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 15 Commerce and Foreign Trade 1 2014-01-01 2014-01-01 false Hearings. 8.12 Section 8.12 Commerce... Compliance § 8.12 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by... hearing, or (2) advise the recipient or other party that the matter in question has been set down for...

15 CFR 8.12 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 15 Commerce and Foreign Trade 1 2011-01-01 2011-01-01 false Hearings. 8.12 Section 8.12 Commerce... Compliance § 8.12 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by... hearing, or (2) advise the recipient or other party that the matter in question has been set down for...

39 CFR 957.13 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 39 Postal Service 1 2012-07-01 2012-07-01 false Hearings. 957.13 Section 957.13 Postal Service... SUSPENSION FROM CONTRACTING § 957.13 Hearings. (a) Hearings are held at 2101 Wilson Boulevard, Suite 600... than 7 days prior to the scheduled date of a hearing, file a request that such hearing be held at a...

12 CFR 308.160 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 12 Banks and Banking 5 2012-01-01 2012-01-01 false Hearings. 308.160 Section 308.160 Banks and... Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 60 days after receipt of a request for hearing on an application filed pursuant to § 308.159. Upon the request...

14 CFR 13.79 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 14 Aeronautics and Space 1 2013-01-01 2013-01-01 false Hearing. 13.79 Section 13.79 Aeronautics....79 Hearing. If an alleged violator requests a hearing in accordance with § 13.75, the procedure of subpart D of this part applies. At the close of the hearing, the Hearing Officer, on the record or...

45 CFR 16.11 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 45 Public Welfare 1 2014-10-01 2014-10-01 false Hearing. 16.11 Section 16.11 Public Welfare... BOARD § 16.11 Hearing. (a) Electing a hearing. If the appellant believes a hearing is appropriate, the... appeal file). The Board will approve a request (and may schedule a hearing on its own or in response to a...

12 CFR 308.160 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 12 Banks and Banking 4 2011-01-01 2011-01-01 false Hearings. 308.160 Section 308.160 Banks and... Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 60 days after receipt of a request for hearing on an application filed pursuant to § 308.159. Upon the request...

39 CFR 957.13 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 957.13 Section 957.13 Postal Service... SUSPENSION FROM CONTRACTING § 957.13 Hearings. (a) Hearings are held at 2101 Wilson Boulevard, Suite 600... than 7 days prior to the scheduled date of a hearing, file a request that such hearing be held at a...

12 CFR 308.155 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 12 Banks and Banking 5 2012-01-01 2012-01-01 false Hearing. 308.155 Section 308.155 Banks and... Pursuant to Section 32 of the FDIA § 308.155 Hearing. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for a hearing filed pursuant...

12 CFR 308.164 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearings. 308.164 Section 308.164 Banks and... Where a Felony ls Charged § 308.164 Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for hearing filed pursuant to § 308.163...

19 CFR 111.67 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 1 2010-04-01 2010-04-01 false Hearing. 111.67 Section 111.67 Customs Duties U.S... Revocation § 111.67 Hearing. (a) Hearing officer. The hearing officer must be an administrative law judge... right to examine all exhibits offered at the hearing and will have the right to cross-examine witnesses...

15 CFR 8.12 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 15 Commerce and Foreign Trade 1 2010-01-01 2010-01-01 false Hearings. 8.12 Section 8.12 Commerce... Compliance § 8.12 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by... hearing, or (2) advise the recipient or other party that the matter in question has been set down for...

12 CFR 308.155 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearing. 308.155 Section 308.155 Banks and... Pursuant to Section 32 of the FDIA § 308.155 Hearing. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 30 days after receipt of a request for a hearing filed pursuant...

12 CFR 308.160 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 12 Banks and Banking 4 2010-01-01 2010-01-01 false Hearings. 308.160 Section 308.160 Banks and... Hearings. (a) Hearing dates. The Executive Secretary shall order a hearing to be commenced within 60 days after receipt of a request for hearing on an application filed pursuant to § 308.159. Upon the request...

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss.

PubMed

Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2013-10-01

Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing.

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

PubMed Central

Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2013-01-01

Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing. PMID:25992037

Hear, Hear!

ERIC Educational Resources Information Center

Rittner-Heir, Robbin

2000-01-01

Examines the problem of acoustics in school classrooms; the problems it creates for student learning, particularly for students with hearing problems; and the impediments to achieving acceptable acoustical levels for school classrooms. Acoustic guidelines are explored and some remedies for fixing sound problems are highlighted. (GR)

Hearing Impairment and Retirement

PubMed Central

Fischer, Mary E; Cruickshanks, Karen J; Pinto, Alex; Klein, Barbara E K; Klein, Ronald; Dalton, Dayna S

2013-01-01

BACKGROUND Many factors influence the decision to retire including age, insurance and pension availability along with physical and mental health. Hearing impairment may be one such factor. PURPOSE The purpose of this study was to compare the 15 year retirement rate among subjects with and without hearing impairment. RESEARCH DESIGN Prospective, population-based study STUDY SAMPLE Subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a longitudinal investigation of age-related hearing loss. Participants who were working full- or part-time in 1993–1995 were included (n=1410, mean age=57.8 years). DATA COLLECTION AND ANALYSIS Data from four EHLS phases (1993–1995, 1998–2000, 2003–2005, and 2009–2010) were analyzed in 2010–2012. Hearing impairment was defined as a pure tone threshold average (at 0.5,1,2 and 4 kHz) greater than 25 dB HL in the worse ear. Employment status was determined at each of the four phases. Kaplan-Meier estimates of the cumulative incidence of retirement were calculated and Cox discrete-time modeling was used to determine the effect of hearing impairment on the rate of retirement. RESULTS The cumulative incidence of retirement was significantly (p < 0.02) higher in those with a hearing impairment (77%) compared to those without a hearing impairment (74%). After adjustment for age, gender, self-reported health, and history of chronic disease, there was no significant difference in the rate of retirement between those with and without a hearing impairment (Hazard Ratio (HR) = 0.9, 95% Confidence Interval (CI) = 0.7, 1.1). Similar results were observed when hearing aid users were excluded, when hearing impairment was based on the better ear thresholds, and when analyses were restricted to those less than 65 years of age and working full-time at baseline. Participants with a hearing impairment were less likely to state that the main reason for retirement was that the time seemed right. CONCLUSIONS Hearing impairment

Cochlear implantation: is hearing preservation necessary in severe to profound hearing loss?

PubMed

Derinsu, Ufuk; Serin, Gediz Murat; Akdaş, Ferda; Batman, Çağlar

2011-03-01

The goal of the cochlear implant surgery is to place the electrode array with minimal damage to preserve the residual hearing. Round-window insertion can be performed in a manner that is potentially less traumatic than the standard cochleostomy. The purpose of the study was to investigate audiological results of the round-window approach using standard electrode. A retrospective study was performed to evaluate our experience in patients with implanted through round window between January 2007 and March 2009. Sixty patients had undergone cochlear implant surgery through the round window with full insertion of a standard electrode array. Preoperative and postoperative pure-tone thresholds were measured for implanted ears in the range of 250 to 4000 Hz. Within these 60 cases, 31 patients had been evaluated. The population comprised 16 women and 15 men. The mean age was 15.96 years (range, 4-64 years). Follow-up times ranged from 6 to 26 months. Preservation of low-frequency hearing (250 and 500 Hz) was achieved in 27 (87%) of 31 patients. Complete hearing preservation (all frequencies) was accomplished in 11 patients (35.48%). No hearing could be determined postoperatively in 4 patients (12.9%), having preoperative thresholds of 120 dB at 250, 500, and 1000 Hz. Round-window approach has been widely used for preservation of residual hearing. In our patients with severe to profound hearing loss, we preserved residual hearing. Although the residual hearing cannot be sufficient for using additional acoustic stimulation, the preserved residual hearing means minimal damage and a more convenient cochlea, so this is promising for future development.

Types of Hearing Aids

MedlinePlus

... hearing impairment. Most hearing aids share several similar electronic components, including a microphone that picks up sound; ... the ear canal; and batteries that power the electronic parts. Hearing aids differ by: design technology used ...

Hearing Disorders and Deafness

MedlinePlus

... enough to enjoy talking with friends or family. Hearing disorders make it hard, but not impossible, to ... often be helped. Deafness can keep you from hearing sound at all. What causes hearing loss? Some ...

39 CFR 964.4 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 964.4 Section 964.4 Postal Service... DELIVERY PURSUANT TO 39 U.S.C. 3003, 3004 § 964.4 Hearings. Hearings are held at 2101 Wilson Boulevard... than 10 days prior to the date fixed for the hearing, a party may file a request that a hearing be held...

39 CFR 964.4 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 39 Postal Service 1 2012-07-01 2012-07-01 false Hearings. 964.4 Section 964.4 Postal Service... DELIVERY PURSUANT TO 39 U.S.C. 3003, 3004 § 964.4 Hearings. Hearings are held at 2101 Wilson Boulevard... than 10 days prior to the date fixed for the hearing, a party may file a request that a hearing be held...

39 CFR 964.4 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 39 Postal Service 1 2013-07-01 2013-07-01 false Hearings. 964.4 Section 964.4 Postal Service... DELIVERY PURSUANT TO 39 U.S.C. 3003, 3004 § 964.4 Hearings. Hearings are held at 2101 Wilson Boulevard... than 10 days prior to the date fixed for the hearing, a party may file a request that a hearing be held...

Does tinnitus, hearing asymmetry, or hearing loss predispose to occupational injury risk?

PubMed

Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Tessier-Sherman, Baylah; Slade, Martin D; Rabinowitz, Peter M; Neitzel, Richard L

2015-02-01

To determine the relative contributions of tinnitus, asymmetrical hearing loss, low frequency hearing loss (pure tone average of 0.5, 1, 2, 3 kHz; PTA.5123), or high frequency hearing loss (pure tone average of 4, 6 kHz; PTA46), to acute injury risk among a cohort of production and maintenance workers at six aluminum manufacturing plants, adjusting for ambient noise exposure and other recognized predictors of injury risk. Retrospective analysis. The study considered 9920 workers employed during 2003 to 2008. The cohort consisted of 8818 workers (89%) whose complete records were available. Adjusting for noise exposure and other recognized injury predictors, a 25% increased acute injury risk was observed among workers with a history of tinnitus in conjunction with high-frequency hearing loss (PTA46). Low frequency hearing loss may be associated with minor, yet less serious, injury risk. We did not find evidence that asymmetry contributes to injury risk. These results provide evidence that tinnitus, combined with high-frequency hearing loss, may pose an important safety threat to workers, especially those who work in high-noise exposed environments. These at risk workers may require careful examination of their communication and hearing protection needs.

High-Level Psychophysical Tuning Curves: Forward Masking in Normal-Hearing and Hearing-Impaired Listeners.

ERIC Educational Resources Information Center

Nelson, David A.

1991-01-01

Forward-masked psychophysical tuning curves were obtained at multiple probe levels from 26 normal-hearing listeners and 24 ears of 21 hearing-impaired listeners with cochlear hearing loss. Results indicated that some cochlear hearing losses influence the sharp tuning capabilities usually associated with outer hair cell function. (Author/JDD)

Buying a Hearing Aid

MedlinePlus

... hearing aids? Federal regulation prohibits any hearing aid sale unless the buyer has first received a physician’s ... care of your hearing aids and other helpful strategies. How should I begin wearing the aids? Start ...

40 CFR 57.804 - Request for hearing; request to participate in hearing.

Code of Federal Regulations, 2010 CFR

2010-07-01

... 40 Protection of Environment 5 2010-07-01 2010-07-01 false Request for hearing; request to participate in hearing. 57.804 Section 57.804 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... of Continuous Emission Reduction Technology § 57.804 Request for hearing; request to participate in...

Factors Influencing Help Seeking, Hearing Aid Uptake, Hearing Aid Use and Satisfaction With Hearing Aids: A Review of the Literature

PubMed Central

Knudsen, Line Vestergaard; Öberg, Marie; Nielsen, Claus; Naylor, Graham; Kramer, Sophia E.

2010-01-01

Objectives: This descriptive summary of the literature provides an overview of the available studies (published between January 1980 and January 2009) on correlates of help-seeking behavior for hearing loss, hearing-aid uptake, hearing-aid use, and satisfaction with the device. Methods: Publications were identified by structured searches in Pubmed and Cinahl and by inspecting the reference lists of relevant articles. The articles covered different stages that a person with hearing impairment may go through: prior to hearing aid fitting, the period covering the fitting and the period post hearing aid fitting. Inclusion of articles occurred according to strict inclusion and exclusion criteria. Data were extracted by two independent researchers. Thirty-nine papers were included that identified 31 factors examined in relation to the four outcome measures. These covered personal factors (e.g., source of motivation, expectation, attitude), demographic factors (e.g., age, gender) and external factors (e.g., cost, counseling). Only two studies covered the actual fitting process. There was only one factor positively affecting all four outcome variables. This was self-reported hearing disability. The vast majority of studies showed no relationship of age and gender with any of the outcome domains. Discussion and conclusion: Whereas research of the last 28 years yielded valuable information regarding relevant and irrelevant factors in hearing aid health care, there are still many relevant issues that have never been investigated in controlled studies. These are discussed. PMID:21109549

Hearing Problems in Children

MedlinePlus

Most children hear and listen from the moment they are born. They learn to talk by imitating the sounds around them ... United States are born deaf or hard-of-hearing. More lose their hearing later during childhood. Babies ...

10 CFR 710.25 - Appointment of Hearing Officer; prehearing conference; commencement of hearings.

Code of Federal Regulations, 2010 CFR

2010-01-01

... papers, issuing subpoenas for witnesses to attend the hearing or for the production of specific documents... extension of the hearing date past 90 calendar days from the date the request for hearing is received by the...

Public hearing on Transbus : stenographic transcript of hearings in the matter of public hearing on Transbus

DOT National Transportation Integrated Search

1977-03-15

Comprises pages 1-212 of a transcript of the public hearing on Transbus, held March 15, 1977, in Washington, D. C., Mortimer L. Downey III, Deputy Undersecretary, U. S. Dept. of Transportation, presiding. The objective of the hearings is to receive i...

78 FR 22546 - Hearing Procedures

Federal Register 2010, 2011, 2012, 2013, 2014

2013-04-16

... FINANCIAL STABILITY OVERSIGHT COUNCIL Hearing Procedures AGENCY: Financial Stability Oversight... Council (Council) has adopted amendments to its hearing procedures (Council Hearing Procedures) for hearings conducted by the Council under Title I and Title VIII of the Dodd-Frank Wall Street Reform and...

Prevention of the Evolution of Workers' Hearing Loss from Noise-Induced Hearing Loss in Noisy Environments through a Hearing Conservation Program

PubMed Central

Fonseca, Vinicius Ribas; Marques, Jair; Panegalli, Flavio; Gonçalves, Claudia Giglio de Oliveira; Souza, Wesley

2015-01-01

Introduction Noise-induced hearing loss (NIHL) is a serious problem for workers and therefore for businesses. The hearing conservation program (HCP) is a set of coordinated measures to prevent the development or evolution of occupational hearing loss, which involves a continuous and dynamic process of implementation of hearing conservation routines through anticipation, recognition, evaluation, and subsequent control of the occurrence of existing environmental risks or of those that may exist in the workplace and lead to workers' hearing damage. Objective The aim of this study was to evaluate the effectiveness of the HCP in preventing further hearing loss in workers with audiograms suggestive of NIHL. The audiometric tests and medical records of 28 furniture company workers exposed to noise were reviewed and monitored for 2 years. Methods This retrospective, cross-sectional study examined five audiometric tests in the medical records (on admission and every semester) of 28 workers in a furniture company (totaling 140 audiometric exams) following the introduction of the HCP. Results Data analysis showed no differences between the audiometric tests conducted on admission and those performed every semester. Conclusions The HCP implemented was effective in preventing the worsening of hearing loss in workers already with NIHL when exposed to occupational noise. Therefore, such a measure could be useful for the employment of workers with hearing loss in job sectors that have noise exposure. PMID:26722345

Hearing is Believing

NASA Technical Reports Server (NTRS)

2003-01-01

This paper presents a discussion on the cochlear implant. This device was developed by Adam Kissiah, who suffers from hearing loss. Driven by his own hearing problem and three failed corrective surgeries, Kissiah started working in the mid-1970s on this surgically implantable device that provides hearing sensation to persons with severe-to-profound hearing loss who receive little or no benefit from hearing aids. Uniquely, the cochlear implant concept was not based on theories of medicine, as Kissiah had no medical background whatsoever. Instead, he utilized the technical expertise he learned while working as an electronics instrumentation engineer at NASA s Kennedy Space Center for the basis of his invention. This took place over 3 years, when Kissiah would spend his lunch breaks and evenings in Kennedy s technical library, studying the impact of engineering principles on the inner ear. In April of 2003, Kissiah was inducted into the Space Foundation's U.S. Space Technology Hall of Fame for his invention

42 CFR 431.222 - Group hearings.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 42 Public Health 4 2012-10-01 2012-10-01 false Group hearings. 431.222 Section 431.222 Public... Beneficiaries Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases in...

42 CFR 431.222 - Group hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 42 Public Health 4 2014-10-01 2014-10-01 false Group hearings. 431.222 Section 431.222 Public... Beneficiaries Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases in...

42 CFR 431.222 - Group hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 42 Public Health 4 2013-10-01 2013-10-01 false Group hearings. 431.222 Section 431.222 Public... Beneficiaries Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases in...

42 CFR 431.222 - Group hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 42 Public Health 4 2011-10-01 2011-10-01 false Group hearings. 431.222 Section 431.222 Public... Recipients Right to Hearing § 431.222 Group hearings. The agency— (a) May respond to a series of individual requests for hearing by conducting a single group hearing; (b) May consolidate hearings only in cases in...

[Rock music and hearing disorders].

PubMed

Størmer, Carl Christian Lein; Stenklev, Niels Christian

2007-03-29

Continued exposition to loud noise is a well-known risk factor for development of various hearing disorders; rock musicians are especially vulnerable. The aim of this paper was to get an overview of hearing loss, tinnitus and hyperacusis among rock musicians. Medline was systematically searched, using combinations of the terms "hearing", "rock music", "tinnitus" and "hyperacusis". Seven publications concerning hearing of rock musicians were identified. Permanent hearing loss occurred in 20% (mean) of the rock musicians; the prevalence varied from 5 to 41%. Tinnitus and hyperacusis appear significantly more often in rock musicians than in non-musicians. Rock musicians have increased resistance against loud music and exposure over time is protective towards hearing loss. Further research is needed to assess rock music's impact on musicians' hearing.

Speech Intelligibility in Persian Hearing Impaired Children with Cochlear Implants and Hearing Aids.

PubMed

Rezaei, Mohammad; Emadi, Maryam; Zamani, Peyman; Farahani, Farhad; Lotfi, Gohar

2017-04-01

The aim of present study is to evaluate and compare speech intelligibility in hearing impaired children with cochlear implants (CI) and hearing aid (HA) users and children with normal hearing (NH). The sample consisted of 45 Persian-speaking children aged 3 to 5-years-old. They were divided into three groups, and each group had 15, children, children with CI and children using hearing aids in Hamadan. Participants was evaluated by the test of speech intelligibility level. Results of ANOVA on speech intelligibility test showed that NH children had significantly better reading performance than hearing impaired children with CI and HA. Post-hoc analysis, using Scheffe test, indicated that the mean score of speech intelligibility of normal children was higher than the HA and CI groups; but the difference was not significant between mean of speech intelligibility in children with hearing loss that use cochlear implant and those using HA. It is clear that even with remarkabkle advances in HA technology, many hearing impaired children continue to find speech production a challenging problem. Given that speech intelligibility is a key element in proper communication and social interaction, consequently, educational and rehabilitation programs are essential to improve speech intelligibility of children with hearing loss.

8 CFR 246.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 8 Aliens and Nationality 1 2012-01-01 2012-01-01 false Hearing. 246.5 Section 246.5 Aliens and... Hearing. (a) Service counsel. The Government shall be represented at the hearing by a Service counsel who... attend or remain in attendance at the hearing. ...

Long-term results of hearing preservation cochlear implant surgery in patients with residual low frequency hearing.

PubMed

Moteki, Hideaki; Nishio, Shin-Ya; Miyagawa, Maiko; Tsukada, Keita; Iwasaki, Satoshi; Usami, Shin-Ichi

2017-05-01

Differences were found between patients with stable hearing and those with progressive hearing loss in the lower frequencies with respect to the rate of progression in the contralateral ear. It is suggested that the electric acoustic stimulation (EAS) can provide improvement in hearing ability over the long-term if residual hearing might be lost to some extent. To evaluate the long-term threshold changes in the low frequency hearing of the implanted ear as compared with the non-implanted ear, and the hearing abilities with EAS along with the extent of residual hearing. Seventeen individuals were enrolled and received the EAS implant with a 24-mm FLEXeas electrode array. Hearing thresholds and speech perception were measured pre- and post-operatively for 1-5 years. Post-operative hearing preservation (HP) rates were calculated using the preservation numerical scale. The average linear regression coefficient for the decline in hearing preservation score was -6.9 for the implanted ear and the patients were subsequently categorized into two groups: those with better than average, stable hearing; and those with worse than average, progressive hearing loss. EAS showed better results than electric stimulation alone, in spite of an absence of speech perception with acoustic stimulation.

Hearing Conservation Medical Program

NASA Technical Reports Server (NTRS)

1993-01-01

Background on hearing impairment is presented including causes and criteria for safe noise levels. The purpose of the Hearing Conservation Program at LeRC is outlined, and the specifics of the Medical Surveillance Program for Hearing Impairment at LeRC are discussed.

Does Tinnitus, Hearing Asymmetry or Hearing Loss Predispose to Occupational Injury Risk?

PubMed Central

Cantley, Linda F; Galusha, Deron; Cullen, Mark R; Dixon-Ernst, Christine; Tessier-Sherman, Baylah; Slade, Martin D; Rabinowitz, Peter M; Neitzel, Richard L

2015-01-01

Objective To determine the relative contributions of tinnitus, asymmetrical hearing loss, low frequency hearing loss (pure tone average of .5, 1, 2, 3 kHz, PTA.5123), or high frequency hearing loss (pure tone average of 4, 6 kHz, PTA46), to acute injury risk among a cohort of production and maintenance workers at six aluminum manufacturing plants, adjusting for ambient noise exposure and other recognized predictors of injury risk. Design and Study Sample This retrospective analysis considered 9,920 workers employed during 2003 to 2008. The cohort consisted of 8,818 workers (89%) whose complete records were available. Results Adjusting for noise exposure and other recognized injury predictors, a 25% increased acute injury risk was observed among workers with a history of tinnitus in conjunction with high-frequency hearing loss (PTA46). Low frequency hearing loss may be associated with minor, yet less serious, injury risk. We did not find evidence that asymmetry contributes to injury risk. Conclusion These results provide evidence that tinnitus, combined with high-frequency hearing loss, may pose an important safety threat to workers, especially those who work in high-noise exposed environments. These at risk workers may require careful examination of their communication and hearing protection needs. PMID:25549168

Hearing Screening Follow-Up: Completing the Process to Identify Hearing Health Needs

ERIC Educational Resources Information Center

Eiserman, William; Shisler, Lenore; Hoffman, Jeff

2015-01-01

Hearing is at the heart of language development and school readiness; increasing numbers of Early Head Start programs have come to rely on otoacoustic emissions (OAE) technology to screen all infants and toddlers for hearing loss. Successful identification of hearing health needs is dependent not only on an appropriate screening method, but also…

Internet Competency Predicts Practical Hearing Aid Knowledge and Skills in First-Time Hearing Aid Users.

PubMed

Maidment, David; Brassington, William; Wharrad, Heather; Ferguson, Melanie

2016-10-01

The purpose of the study was to assess whether Internet competency predicted practical hearing aid knowledge and handling skills in first-time hearing aid users. The design was a prospective, randomized controlled trial of a multimedia educational intervention consisting of interactive video tutorials (or reusable learning objects [RLOs]). RLOs were delivered through DVD for TV or PC, and online. Internet competency was measured at the hearing aid fitting appointment, whereas hearing aid knowledge and practical handling skills were assessed 6 weeks postfitting. Internet competency predicted practical hearing aid knowledge and handling skills, controlling for age, hearing sensitivity, educational status, and gender for the group that received the RLOs. Internet competency was inversely related to the number of times the RLOs were watched. Associations between Internet competency and practical hearing aid knowledge, handling skills, and watching the RLOs fewer times may have arisen because of improved self-efficacy. Therefore, first-time hearing aid users who are more competent Internet users may be better equipped to apply newly learned information to effectively manage their hearing loss.

Validity, Discriminative Ability and Reliability of the Hearing-Related Quality of Life (HEAR-QL) Questionnaire for Adolescents

PubMed Central

Rachakonda, Tara; Jeffe, Donna B.; Shin, Jennifer J.; Mankarious, Leila; Fanning, Robert J.; Lesperance, Marci M.; Lieu, Judith E.C.

2014-01-01

Objectives The prevalence of hearing loss (HL) in adolescents has grown over the past decade, but hearing-related quality of life (QOL) has not been well-measured. We sought to develop a reliable, valid measure of hearing-related QOL for adolescents, the Hearing Environments And Reflection on Quality of Life (HEAR-QL). Study Design Multi-site observational study. Methods Adolescents with HL and siblings without HL were recruited from five centers. Participants completed the HEAR-QL and validated questionnaires measuring generic pediatric QOL (PedsQL), depression and anxiety (RCADS-25), and hearing-related QOL for adults (HHIA) to determine construct and discriminant validity. Participants completed the HEAR-QL two weeks later for test-retest reliability. We used exploratory principal components analysis to determine the HEAR-QL factor structure and measured reliability. Sensitivity and specificity of the HEAR-QL, PedsQL, HHIA and RCADS-25 were assessed. We compared scores on all surveys between those with normal hearing, unilateral and bilateral HL. Results 233 adolescents (13–18 years old) participated—179 with HL, 54 without HL. The original 45-item HEAR-QL was shortened to 28 items after determining factor structure. The resulting HEAR-QL-28 demonstrated excellent reliability (Cronbach’s alpha= 0.95) and construct validity (HHIA: r =.845, PedsQL: r =.587; RCADS-25: r =.433). The HEAR-QL-28 displayed excellent discriminant validity, with higher area under the curve (0.932) than the PedsQL (0.597) or RCADS-25 (0.529). Teens with bilateral HL using hearing devices reported worse QOL on the HEAR-QL and HHIA than peers with HL not using devices. Conclusions The HEAR-QL is a sensitive, reliable and valid measure of hearing-related QOL for adolescents. PMID:23900836

Self-Assessment of Hearing and Purchase of Hearing Aids by Middle-Aged and Elderly Adults

PubMed Central

Otavio, Andressa Colares da Costa; Coradini, Patricia Pérez; Teixeira, Adriane Ribeiro

2015-01-01

Introduction Presbycusis is a consequence of aging. Prescription of hearing aids is part of the treatment, although the prevalence of use by elderly people is still small. Objective To verify whether or not self-assessment of hearing is a predictor for purchase of hearing aids. Methods Quantitative, cross-sectional, descriptive, and observational study. Participants were subjects who sought a private hearing center for selection of hearing aids. During the diagnostic interview, subjects answered the following question: “On a scale of 1 to 10, with 1 being the worst and 10 the best, how would you rate your overall hearing ability?” After that, subjects underwent audiometry, selected a hearing aid, performed a home trial, and decided whether or not to purchase the hearing aid. The variables were associated and analyzed statistically. Results The sample was comprised of 32 subjects, both men and women, with a higher number of women. Mean age was 71.41 ± 12.14 years. Self-assessment of hearing ranged from 2 to 9 points. Overall, 71.9% of the subjects purchased hearing aids. There was no association between scores in the self-assessment and the purchase of hearing aids (p = 0.263). Among those who scored between 2 and 5 points, 64.7% purchased the device; between 6 and 7 points, 76.09% purchased the device; and between 8 and 9 points, 50% purchased the device, respectively. Conclusion There is evidence that low self-assessment scores lead to the purchase of hearing aids, although no significant association was observed in the sample. PMID:26722346

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree.

PubMed

Fu, Siqing; Yan, Ju; Wang, Xiyin; Dong, Jiashu; Chen, Peiwei; Wang, Chunfang; Chen, Guanming

2011-02-01

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type. Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15dB hearing loss averaged over 0.5, 1 and 2kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4kHz, and classified into four categories: mild, moderate, severe and profound. Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss. The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

39 CFR 775.14 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 39 Postal Service 1 2012-07-01 2012-07-01 false Hearings. 775.14 Section 775.14 Postal Service....14 Hearings. (a) Public hearings must be held whenever there is: (1) Substantial environmental controversy concerning a proposed action and a request for a hearing by any responsible individual or...

39 CFR 775.14 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 39 Postal Service 1 2013-07-01 2013-07-01 false Hearings. 775.14 Section 775.14 Postal Service....14 Hearings. (a) Public hearings must be held whenever there is: (1) Substantial environmental controversy concerning a proposed action and a request for a hearing by any responsible individual or...

39 CFR 775.14 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 39 Postal Service 1 2011-07-01 2011-07-01 false Hearings. 775.14 Section 775.14 Postal Service....14 Hearings. (a) Public hearings must be held whenever there is: (1) Substantial environmental controversy concerning a proposed action and a request for a hearing by any responsible individual or...

24 CFR 30.95 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 24 Housing and Urban Development 1 2011-04-01 2011-04-01 false Hearings. 30.95 Section 30.95... MONEY PENALTIES: CERTAIN PROHIBITED CONDUCT Procedures § 30.95 Hearings. Hearings under this part shall be conducted in accordance with the procedures applicable to hearings in accordance with the...

49 CFR 209.321 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 4 2011-10-01 2011-10-01 false Hearing. 209.321 Section 209.321 Transportation... TRANSPORTATION RAILROAD SAFETY ENFORCEMENT PROCEDURES Disqualification Procedures § 209.321 Hearing. (a) Upon receipt of a hearing request complying with § 209.311, an administrative hearing for review of a notice of...

49 CFR 240.409 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 4 2011-10-01 2011-10-01 false Hearings. 240.409 Section 240.409 Transportation... Hearings. (a) An administrative hearing for a locomotive engineer qualification petition shall be conducted... conduct of the hearing for the purpose of achieving a prompt and fair determination of all material issues...

45 CFR 400.23 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 45 Public Welfare 2 2014-10-01 2012-10-01 true Hearings. 400.23 Section 400.23 Public Welfare....23 Hearings. (a) A State must provide applicants for, and recipients of, assistance and services under the Act with an opportunity for a hearing to contest adverse determinations using hearing...

24 CFR 30.95 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 24 Housing and Urban Development 1 2013-04-01 2013-04-01 false Hearings. 30.95 Section 30.95... MONEY PENALTIES: CERTAIN PROHIBITED CONDUCT Procedures § 30.95 Hearings. Hearings under this part shall be conducted in accordance with the procedures applicable to hearings in accordance with the...

24 CFR 30.95 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 24 Housing and Urban Development 1 2014-04-01 2014-04-01 false Hearings. 30.95 Section 30.95... MONEY PENALTIES: CERTAIN PROHIBITED CONDUCT Procedures § 30.95 Hearings. Hearings under this part shall be conducted in accordance with the procedures applicable to hearings in accordance with the...

49 CFR 240.409 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 49 Transportation 4 2010-10-01 2010-10-01 false Hearings. 240.409 Section 240.409 Transportation... Hearings. (a) An administrative hearing for a locomotive engineer qualification petition shall be conducted... conduct of the hearing for the purpose of achieving a prompt and fair determination of all material issues...

49 CFR 209.321 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 49 Transportation 4 2010-10-01 2010-10-01 false Hearing. 209.321 Section 209.321 Transportation... TRANSPORTATION RAILROAD SAFETY ENFORCEMENT PROCEDURES Disqualification Procedures § 209.321 Hearing. (a) Upon receipt of a hearing request complying with § 209.311, an administrative hearing for review of a notice of...

5 CFR 831.1106 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Hearing. 831.1106 Section 831.1106...) RETIREMENT Prohibition on Payments of Annuities § 831.1106 Hearing. (a) OPM's hearing examiner shall preside at any hearing held pursuant to this subpart, unless OPM designates another presiding officer. The...

The Stigma of Hearing Loss

PubMed Central

Wallhagen, Margaret I.

2010-01-01

Purpose: To explore dimensions of stigma experienced by older adults with hearing loss and those with whom they frequently communicate to target interventions promoting engagement and positive aging. Design and Methods: This longitudinal qualitative study conducted interviews over 1 year with dyads where one partner had hearing loss. Participants were naive to or had not worn hearing aids in the past year. Data were analyzed using grounded theory, constant comparative methodology. Results: Perceived stigma emerged as influencing decision-making processes at multiple points along the experiential continuum of hearing loss, such as initial acceptance of hearing loss, whether to be tested, type of hearing aid selected, and when and where hearing aids were worn. Stigma was related to 3 interrelated experiences, alterations in self-perception, ageism, and vanity and was influenced by dyadic relationships and external societal forces, such as health and hearing professionals and media. Implications: Findings are discussed in relation to theoretical perspectives regarding stigma and ageism and suggest the need to destigmatize hearing loss by promoting its assessment and treatment as well as emphasizing the importance of remaining actively engaged to support positive physical and cognitive functioning. PMID:19592638

Three novel GJB2 (connexin 26) variants associated with autosomal dominant syndromic and nonsyndromic hearing loss.

PubMed

DeMille, Desiree; Carlston, Colleen M; Tam, Oliver H; Palumbos, Janice C; Stalker, Heather J; Mao, Rong; Zori, Roberto T; Viskochil, David H; Park, Albert H; Carey, John C

2018-04-01

Connexin 26 (Cx26), encoded by the GJB2 gene, is a key protein involved in the formation of gap junctions in epithelial organs including the inner ear and palmoplantar epidermis. Pathogenic variants in GJB2 are responsible for approximately 50% of inherited sensorineural deafness. The majority of these variants are associated with autosomal recessive inheritance; however, rare reports of dominantly co-segregating variants have been published. Since we began offering GJB2 testing in 2003, only about 2% of detected GJB2 variants from our laboratory have been classified as dominant. Here we report three novel dominant GJB2 variants (p.Thr55Ala, p.Gln57_Pro58delinsHisSer, and p.Trp44Gly); two associated with syndromic sensorineural hearing loss and one with nonsyndromic hearing loss. In the kindred with the p.Thr55Ala variant, the proband and his father present with only leukonychia as a cutaneous finding of their syndromic hearing loss. This phenotype has been previously documented in conjunction with palmoplantar hyperkeratosis, but isolated leukonychia is a novel finding likely associated with the unique threonine to alanine change at codon 55 (other variants at this codon have been reported in cases of nonsyndromic hearing loss). This report contributes to the short list of GJB2 variants associated with autosomal dominant hearing loss, highlights the variability of skin and nail findings associated with such cases, and illustrates the occurrence of both syndromic and nonsyndromic presentations with changes in the same gene. © 2018 Wiley Periodicals, Inc.

49 CFR 27.127 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 49 Transportation 1 2012-10-01 2012-10-01 false Hearings. 27.127 Section 27.127 Transportation... ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Enforcement § 27.127 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 27.125(b), reasonable notice is given by the...

15 CFR 90.14 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 15 Commerce and Foreign Trade 1 2011-01-01 2011-01-01 false Hearing. 90.14 Section 90.14 Commerce... OF COMMERCE PROCEDURE FOR CHALLENGING CERTAIN POPULATION AND INCOME ESTIMATES § 90.14 Hearing. (a) The hearing shall be conducted by the same hearing officer who collected the documentary evidence, if...

29 CFR 1921.12 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 7 2013-07-01 2013-07-01 false Hearing. 1921.12 Section 1921.12 Labor Regulations Relating...' COMPENSATION ACT Hearing and Related Matters § 1921.12 Hearing. (a) Order of proceeding; burden of proof. Except as may be determined otherwise by the hearing examiner, counsel supporting the complaint shall...

24 CFR 81.84 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 24 Housing and Urban Development 1 2011-04-01 2011-04-01 false Hearings. 81.84 Section 81.84... LOAN MORTGAGE CORPORATION (FREDDIE MAC) Procedures for Actions and Review of Actions § 81.84 Hearings. (a) Applicability. The hearing procedures in this section apply to hearings on the record to review...

39 CFR 952.14 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 39 Postal Service 1 2012-07-01 2012-07-01 false Hearings. 952.14 Section 952.14 Postal Service... AND LOTTERY ORDERS § 952.14 Hearings. Hearings are held at 2101 Wilson Boulevard, Suite 600, Arlington... the hearing shall be set by the presiding officer in his or her sole discretion. ...

39 CFR 952.14 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 39 Postal Service 1 2013-07-01 2013-07-01 false Hearings. 952.14 Section 952.14 Postal Service... AND LOTTERY ORDERS § 952.14 Hearings. Hearings are held at 2101 Wilson Boulevard, Suite 600, Arlington... the hearing shall be set by the presiding officer in his or her sole discretion. ...

24 CFR 81.84 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 24 Housing and Urban Development 1 2013-04-01 2013-04-01 false Hearings. 81.84 Section 81.84... LOAN MORTGAGE CORPORATION (FREDDIE MAC) Procedures for Actions and Review of Actions § 81.84 Hearings. (a) Applicability. The hearing procedures in this section apply to hearings on the record to review...

22 CFR 18.15 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 22 Foreign Relations 1 2014-04-01 2014-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open...

22 CFR 18.15 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 22 Foreign Relations 1 2013-04-01 2013-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open...

39 CFR 953.5 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 39 Postal Service 1 2013-07-01 2013-07-01 false Hearings. 953.5 Section 953.5 Postal Service... Hearings. (a) In general, admissibility of evidence at hearings conducted under this part hinges on... the rulings of the presiding officer made during the course of the hearing are unnecessary. For all...

22 CFR 18.15 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 22 Foreign Relations 1 2011-04-01 2011-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open...

49 CFR 211.25 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 49 Transportation 4 2011-10-01 2011-10-01 false Hearings. 211.25 Section 211.25 Transportation... TRANSPORTATION RULES OF PRACTICE Rulemaking Procedures § 211.25 Hearings. (a) A hearing will be held if required by statute or the Administrator finds it necessary or desirable. (b) Except for statutory hearings...

47 CFR 1.1928 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 47 Telecommunication 1 2011-10-01 2011-10-01 false Hearing. 1.1928 Section 1.1928... United States Salary Offset-Individual Debt § 1.1928 Hearing. (a) Petition for hearing. (1) An employee may request a hearing by filing a written petition with the Managing Director of the Commission, or...

24 CFR 81.84 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 24 Housing and Urban Development 1 2014-04-01 2014-04-01 false Hearings. 81.84 Section 81.84... LOAN MORTGAGE CORPORATION (FREDDIE MAC) Procedures for Actions and Review of Actions § 81.84 Hearings. (a) Applicability. The hearing procedures in this section apply to hearings on the record to review...

20 CFR 901.44 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 20 Employees' Benefits 4 2012-04-01 2012-04-01 false Hearings. 901.44 Section 901.44 Employees... § 901.44 Hearings. (a) In general. The Administrative Law Judge shall preside at the hearing on a complaint for the suspension or termination of an enrolled actuary. Hearings shall be stenographically...

49 CFR 27.127 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 49 Transportation 1 2013-10-01 2013-10-01 false Hearings. 27.127 Section 27.127 Transportation... ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Enforcement § 27.127 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 27.125(b), reasonable notice is given by the...

29 CFR 1921.12 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 7 2014-07-01 2014-07-01 false Hearing. 1921.12 Section 1921.12 Labor Regulations Relating...' COMPENSATION ACT Hearing and Related Matters § 1921.12 Hearing. (a) Order of proceeding; burden of proof. Except as may be determined otherwise by the hearing examiner, counsel supporting the complaint shall...

20 CFR 901.44 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-04-01

... 20 Employees' Benefits 3 2011-04-01 2011-04-01 false Hearings. 901.44 Section 901.44 Employees... § 901.44 Hearings. (a) In general. The Administrative Law Judge shall preside at the hearing on a complaint for the suspension or termination of an enrolled actuary. Hearings shall be stenographically...

20 CFR 901.44 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 20 Employees' Benefits 4 2014-04-01 2014-04-01 false Hearings. 901.44 Section 901.44 Employees... § 901.44 Hearings. (a) In general. The Administrative Law Judge shall preside at the hearing on a complaint for the suspension or termination of an enrolled actuary. Hearings shall be stenographically...

29 CFR 1614.109 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 4 2012-07-01 2012-07-01 false Hearings. 1614.109 Section 1614.109 Labor Regulations... OPPORTUNITY Agency Program To Promote Equal Employment Opportunity § 1614.109 Hearings. (a) When a complainant requests a hearing, the Commission shall appoint an administrative judge to conduct a hearing in accordance...

39 CFR 953.5 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 39 Postal Service 1 2012-07-01 2012-07-01 false Hearings. 953.5 Section 953.5 Postal Service... Hearings. (a) In general, admissibility of evidence at hearings conducted under this part hinges on... the rulings of the presiding officer made during the course of the hearing are unnecessary. For all...

29 CFR 1614.109 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 4 2014-07-01 2014-07-01 false Hearings. 1614.109 Section 1614.109 Labor Regulations... OPPORTUNITY Agency Program To Promote Equal Employment Opportunity § 1614.109 Hearings. (a) When a complainant requests a hearing, the Commission shall appoint an administrative judge to conduct a hearing in accordance...

17 CFR 141.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 17 Commodity and Securities Exchanges 1 2012-04-01 2012-04-01 false Hearing. 141.5 Section 141.5 Commodity and Securities Exchanges COMMODITY FUTURES TRADING COMMISSION SALARY OFFSET § 141.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the...

22 CFR 18.15 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 22 Foreign Relations 1 2010-04-01 2010-04-01 false Hearings. 18.15 Section 18.15 Foreign Relations... Enforcement Proceedings § 18.15 Hearings. Hearings shall be stenographically recorded and transcribed and the testimony of witnesses shall be taken under oath or affirmation. Hearings will be closed unless an open...

49 CFR 211.25 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 49 Transportation 4 2010-10-01 2010-10-01 false Hearings. 211.25 Section 211.25 Transportation... TRANSPORTATION RULES OF PRACTICE Rulemaking Procedures § 211.25 Hearings. (a) A hearing will be held if required by statute or the Administrator finds it necessary or desirable. (b) Except for statutory hearings...

15 CFR 90.14 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 15 Commerce and Foreign Trade 1 2010-01-01 2010-01-01 false Hearing. 90.14 Section 90.14 Commerce... OF COMMERCE PROCEDURE FOR CHALLENGING CERTAIN POPULATION AND INCOME ESTIMATES § 90.14 Hearing. (a) The hearing shall be conducted by the same hearing officer who collected the documentary evidence, if...

19 CFR 354.12 - Hearing.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearing. 354.12 Section 354.12 Customs Duties... ANTIDUMPING OR COUNTERVAILING DUTY ADMINISTRATIVE PROTECTIVE ORDER § 354.12 Hearing. (a) Scheduling of hearing. The presiding official will schedule the hearing at a reasonable time, date, and place, which will be...

19 CFR 351.310 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearings. 351.310 Section 351.310 Customs Duties... Argument § 351.310 Hearings. (a) Introduction. This section sets forth the procedures for requesting a hearing, indicates that the Secretary may consolidate hearings, and explains when the Secretary may hold...

19 CFR 207.112 - Hearings.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 19 Customs Duties 3 2010-04-01 2010-04-01 false Hearings. 207.112 Section 207.112 Customs Duties... and Committee Proceedings § 207.112 Hearings. (a) Purpose of and scheduling of hearings. An opportunity for a hearing before an administrative law judge shall be provided for each action initiated under...

Genetic variants in TPMT and COMT are associated with hearing loss in children receiving cisplatin chemotherapy.

PubMed

Ross, Colin J D; Katzov-Eckert, Hagit; Dubé, Marie-Pierre; Brooks, Beth; Rassekh, S Rod; Barhdadi, Amina; Feroz-Zada, Yassamin; Visscher, Henk; Brown, Andrew M K; Rieder, Michael J; Rogers, Paul C; Phillips, Michael S; Carleton, Bruce C; Hayden, Michael R

2009-12-01

Cisplatin is a widely used and effective chemotherapeutic agent, although its use is restricted by the high incidence of irreversible ototoxicity associated with it. In children, cisplatin ototoxicity is a serious and pervasive problem, affecting more than 60% of those receiving cisplatin and compromising language and cognitive development. Candidate gene studies have previously reported associations of cisplatin ototoxicity with genetic variants in the genes encoding glutathione S-transferases and megalin. We report association analyses for 220 drug-metabolism genes in genetic susceptibility to cisplatin-induced hearing loss in children. We genotyped 1,949 SNPs in these candidate genes in an initial cohort of 54 children treated in pediatric oncology units, with replication in a second cohort of 112 children recruited through a national surveillance network for adverse drug reactions in Canada. We identified genetic variants in TPMT (rs12201199, P value = 0.00022, OR = 17.0, 95% CI 2.3-125.9) and COMT (rs9332377, P value = 0.00018, OR = 5.5, 95% CI 1.9-15.9) associated with cisplatin-induced hearing loss in children.

Senate Hearing

NASA Image and Video Library

2012-03-07

Astrophysicist, American Museum of Natural History and Director, Hayden Planetarium Dr. Neil deGrasse Tyson testifies during a U.S. Senate Committee on Commerce, Science, and Transportation hearing on Wednesday, March 7, 2012 in Washington. NASA Administrator Charles Bolden testified at the same hearing prior to Tyson. Photo Credit: (NASA/Bill Ingalls)

29 CFR 1905.26 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 29 Labor 5 2014-07-01 2014-07-01 false Hearings. 1905.26 Section 1905.26 Labor Regulations... OCCUPATIONAL SAFETY AND HEALTH ACT OF 1970 Hearings § 1905.26 Hearings. (a) Order of proceeding. Except as may be ordered otherwise by the presiding hearing examiner, the party applicant for relief shall proceed...

29 CFR 34.51 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 1 2012-07-01 2012-07-01 false Hearings. 34.51 Section 34.51 Labor Office of the Secretary... Hearings. (a) Notice of opportunity for hearing. As part of a Final Determination, or a Notification of... recipient (by certified mail, return receipt requested), a notice of opportunity for hearing. (b) Complaint...

7 CFR 900.115 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 7 Agriculture 8 2011-01-01 2011-01-01 false Hearing. 900.115 Section 900.115 Agriculture... Hearing. (a) The arbitrator shall have full discretion to conduct the hearing in such manner as will, in..., and other experts. (h) When more than two arbitrators are designated to hear a dispute, and they...

5 CFR 1639.23 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 5 Administrative Personnel 3 2014-01-01 2014-01-01 false Hearing. 1639.23 Section 1639.23... Hearing. (a) Request for hearing. Except as provided in paragraph (b) of this section, an employee who desires a hearing concerning the existence or amount of the debt or the proposed offset schedule must send...

45 CFR 33.6 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 45 Public Welfare 1 2014-10-01 2014-10-01 false Hearings. 33.6 Section 33.6 Public Welfare Department of Health and Human Services GENERAL ADMINISTRATION SALARY OFFSET § 33.6 Hearings. (a) Petitions for hearing. (1) To request a hearing concerning the existence or amount of the debt or the offset...

40 CFR 53.13 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 40 Protection of Environment 6 2014-07-01 2014-07-01 false Hearings. 53.13 Section 53.13... MONITORING REFERENCE AND EQUIVALENT METHODS General Provisions § 53.13 Hearings. (a)(1) After granting a request for a hearing under § 53.12, the Administrator will designate a presiding officer for the hearing...

29 CFR 34.51 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 1 2013-07-01 2013-07-01 false Hearings. 34.51 Section 34.51 Labor Office of the Secretary... Hearings. (a) Notice of opportunity for hearing. As part of a Final Determination, or a Notification of... recipient (by certified mail, return receipt requested), a notice of opportunity for hearing. (b) Complaint...

17 CFR 8.17 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 17 Commodity and Securities Exchanges 1 2012-04-01 2012-04-01 false Hearing. 8.17 Section 8.17..., SUMMARY, AND MEMBERSHIP DENIAL ACTIONS Disciplinary Procedure § 8.17 Hearing. (a) The following minimum requirements shall apply to any hearing required by this subpart: (1) The hearing shall be fair and shall be...

45 CFR 96.51 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 96.51 Section 96.51 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION BLOCK GRANTS Enforcement § 96.51 Hearings. (a... notice of the order and an opportunity for a hearing. Opportunity for a hearing will not be provided...

29 CFR 30.16 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 29 Labor 1 2011-07-01 2011-07-01 false Hearings. 30.16 Section 30.16 Labor Office of the Secretary of Labor EQUAL EMPLOYMENT OPPORTUNITY IN APPRENTICESHIP AND TRAINING § 30.16 Hearings. (a) Within 10 days after receiving a request for a hearing, the Secretary shall designate a hearing officer. The...

5 CFR 1639.23 - Hearing.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 5 Administrative Personnel 3 2011-01-01 2011-01-01 false Hearing. 1639.23 Section 1639.23... Hearing. (a) Request for hearing. Except as provided in paragraph (b) of this section, an employee who desires a hearing concerning the existence or amount of the debt or the proposed offset schedule must send...

5 CFR 1639.23 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 5 Administrative Personnel 3 2013-01-01 2013-01-01 false Hearing. 1639.23 Section 1639.23... Hearing. (a) Request for hearing. Except as provided in paragraph (b) of this section, an employee who desires a hearing concerning the existence or amount of the debt or the proposed offset schedule must send...

22 CFR 1007.5 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-04-01

... 22 Foreign Relations 2 2013-04-01 2009-04-01 true Hearing. 1007.5 Section 1007.5 Foreign Relations INTER-AMERICAN FOUNDATION SALARY OFFSET § 1007.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to...

45 CFR 96.51 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 45 Public Welfare 1 2014-10-01 2014-10-01 false Hearings. 96.51 Section 96.51 Public Welfare Department of Health and Human Services GENERAL ADMINISTRATION BLOCK GRANTS Enforcement § 96.51 Hearings. (a... notice of the order and an opportunity for a hearing. Opportunity for a hearing will not be provided...

22 CFR 1007.5 - Hearing.

Code of Federal Regulations, 2014 CFR

2014-04-01

... 22 Foreign Relations 2 2014-04-01 2014-04-01 false Hearing. 1007.5 Section 1007.5 Foreign Relations INTER-AMERICAN FOUNDATION SALARY OFFSET § 1007.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice...

31 CFR 28.620 - Hearings.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 31 Money and Finance: Treasury 1 2014-07-01 2014-07-01 false Hearings. 28.620 Section 28.620 Money... EDUCATION PROGRAMS OR ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Procedures § 28.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 28.615(c), reasonable notice...

16 CFR 1027.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 16 Commercial Practices 2 2012-01-01 2012-01-01 false Hearing. 1027.5 Section 1027.5 Commercial Practices CONSUMER PRODUCT SAFETY COMMISSION GENERAL SALARY OFFSET § 1027.5 Hearing. (a) Request for hearing. (1) An employee may file a petition for an oral or paper hearing in accordance with the instructions...

49 CFR 386.56 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 49 Transportation 5 2013-10-01 2013-10-01 false Hearings. 386.56 Section 386.56 Transportation... and Hearings § 386.56 Hearings. (a) As soon as practicable after his/her appointment, the administrative law judge shall issue an order setting the date, time, and place for the hearing. The order shall...

31 CFR 28.620 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 31 Money and Finance: Treasury 1 2011-07-01 2011-07-01 false Hearings. 28.620 Section 28.620 Money... EDUCATION PROGRAMS OR ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Procedures § 28.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 28.615(c), reasonable notice...

45 CFR 33.6 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 45 Public Welfare 1 2011-10-01 2011-10-01 false Hearings. 33.6 Section 33.6 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION SALARY OFFSET § 33.6 Hearings. (a) Petitions for hearing. (1) To request a hearing concerning the existence or amount of the debt or the offset...

29 CFR 2200.209 - Hearing.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 29 Labor 9 2013-07-01 2013-07-01 false Hearing. 2200.209 Section 2200.209 Labor Regulations... Simplified Proceedings § 2200.209 Hearing. (a) Procedures. As soon as practicable after the conclusion of the pre-hearing conference, the Judge will hold a hearing on any issue that remains in dispute. The...

45 CFR 33.6 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 45 Public Welfare 1 2012-10-01 2012-10-01 false Hearings. 33.6 Section 33.6 Public Welfare DEPARTMENT OF HEALTH AND HUMAN SERVICES GENERAL ADMINISTRATION SALARY OFFSET § 33.6 Hearings. (a) Petitions for hearing. (1) To request a hearing concerning the existence or amount of the debt or the offset...

31 CFR 28.620 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 31 Money and Finance: Treasury 1 2013-07-01 2013-07-01 false Hearings. 28.620 Section 28.620 Money... EDUCATION PROGRAMS OR ACTIVITIES RECEIVING FEDERAL FINANCIAL ASSISTANCE Procedures § 28.620 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 28.615(c), reasonable notice...

45 CFR 1110.9 - Hearings.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 45 Public Welfare 3 2013-10-01 2013-10-01 false Hearings. 1110.9 Section 1110.9 Public Welfare... NONDISCRIMINATION IN FEDERALLY ASSISTED PROGRAMS § 1110.9 Hearings. (a) Opportunity for hearing. Whenever an opportunity for a hearing is required by § 1110.8(c), reasonable notice shall be given by registered or...

29 CFR 2200.209 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 9 2012-07-01 2012-07-01 false Hearing. 2200.209 Section 2200.209 Labor Regulations... Simplified Proceedings § 2200.209 Hearing. (a) Procedures. As soon as practicable after the conclusion of the pre-hearing conference, the Judge will hold a hearing on any issue that remains in dispute. The...

22 CFR 1007.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 22 Foreign Relations 2 2012-04-01 2009-04-01 true Hearing. 1007.5 Section 1007.5 Foreign Relations INTER-AMERICAN FOUNDATION SALARY OFFSET § 1007.5 Hearing. (a) Request for hearing. (1) An employee must file a petition for a hearing in accordance with the instructions outlined in the agency's notice to...

29 CFR 34.51 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 29 Labor 1 2011-07-01 2011-07-01 false Hearings. 34.51 Section 34.51 Labor Office of the Secretary... Hearings. (a) Notice of opportunity for hearing. As part of a Final Determination, or a Notification of... recipient (by certified mail, return receipt requested), a notice of opportunity for hearing. (b) Complaint...

18 CFR 156.10 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-04-01

... 18 Conservation of Power and Water Resources 1 2012-04-01 2012-04-01 false Hearings. 156.10... GAS ACT § 156.10 Hearings. The Commission will schedule each application for public hearing at the... protests or petitions to intervene, issue the requested order without hearing. [Order 234, 26 FR 4848, June...

40 CFR 1042.920 - Hearings.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 40 Protection of Environment 33 2011-07-01 2011-07-01 false Hearings. 1042.920 Section 1042.920... Reference Information § 1042.920 Hearings. (a) You may request a hearing under certain circumstances, as... your objection and any supporting data, within 30 days after we make a decision. (b) For a hearing you...

29 CFR 30.16 - Hearings.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 29 Labor 1 2012-07-01 2012-07-01 false Hearings. 30.16 Section 30.16 Labor Office of the Secretary of Labor EQUAL EMPLOYMENT OPPORTUNITY IN APPRENTICESHIP AND TRAINING § 30.16 Hearings. (a) Within 10 days after receiving a request for a hearing, the Secretary shall designate a hearing officer. The...

32 CFR 1697.5 - Hearing.

Code of Federal Regulations, 2012 CFR

2012-07-01